Common Variable Immunodeficiency
Agammaglobulinemia
Immunologic Deficiency Syndromes
Immunoglobulins, Intravenous
Dysgammaglobulinemia
Transmembrane Activator and CAML Interactor Protein
B-Lymphocytes
Immunoglobulins
B-Lymphocyte Subsets
Aortic Arch Syndromes
Immunoglobulin M
Pseudolymphoma
T-Lymphocytopenia, Idiopathic CD4-Positive
Lymphocyte Activation
Complement C4a
Immunoglobulin Class Switching
Granuloma
Immunoglobulin G
Tumor Necrosis Factor Ligand Superfamily Member 13
T-Lymphocytes
Immunophenotyping
Immunoglobulin A
Opportunistic Infections
HIV-1
Antigens, CD27
Immunologic Memory
Cardiovascular Abnormalities
Interleukin-2
Anemia, Hemolytic, Autoimmune
Lung Diseases, Interstitial
Acquired Immunodeficiency Syndrome
Simian immunodeficiency virus
CD4-CD8 Ratio
Autoimmunity
CD4-Positive T-Lymphocytes
Infection
Antibody Formation
Antigens, CD
T-Lymphocyte Subsets
Flow Cytometry
Respiratory Tract Infections
Lymphoproliferative Disorders
Lymphocyte Subsets
In vivo modulation of cytokine synthesis by intravenous immunoglobulin. (1/340)
We examined the effects of intravenous immunoglobulin (IVIG) on cytokine regulation in vivo using samples taken before and after replacement-dose (200-400 mg/kg) IVIG in a group of patients with common variable immunodeficiency (CVID) and X-linked agammaglobulinaemia (XLA). The intracellular cytokine content of CD4+ and CD8+ lymphocytes, and their CD28+/- subsets, were measured following in vitro activation with phorbol myristate acetate (PMA) and ionomycin. The cytokines IL-2, interferon-gamma (IFN-gamma) and tumour necrosis factor-alpha (TNF-alpha), and the early activation marker CD69, were assessed by four-colour flow cytometry of whole blood cultures taken before and after IVIG infusion. There was a significant increase in IL-2 expression in CD4+ (and CD4+28-) cells and an increase in TNF-alpha expression in CD8+28- cells following IVIG in CVID, but not in XLA patients. IFN-gamma and CD69 expression were not affected by IVIG infusion. This increase in TNF-alpha and IL-2, combined with unchanged IFN-gamma expression, is evidence against the putative 'anti-inflammatory' role of IVIG, and may explain the failure of resolution of granulomata in CVID patients treated with IVIG alone. (+info)HLA class II homozygosity confers susceptibility to common variable immunodeficiency (CVID). (2/340)
Most cases of CVID occur sporadically, but familial cases do also occur and 15% of the patients with the disease have first degree relatives with IgA deficiency (IgAD). Our purpose was to study CVID association with HLA class II alleles and to ascertain whether this disease shares a common genetic background with IgAD in our population. Patients with CVID (n = 42), were typed using gene amplification and sequence-specific oligonucleotide probing for HLA-DRB1, DRB3, DQA1 and DQB1 loci and their typing compared with that of 96 IgAD and 334 healthy controls. We observed a positive association between non-Asp residues at position 57 of the HLA-DQbeta chain and CVID, although much weaker than in IgAD. Further, we found an association between CVID and homozygosity for genes encoding HLA class II molecules, especially HLA-DQ, not seen in IgAD. The data support the hypothesis that a restricted diversity of HLA class II molecules may contribute to susceptibility to CVID. (+info)Gastric pathology in patients with common variable immunodeficiency. (3/340)
BACKGROUND/AIMS: Common variable immunodeficiency (CVID) is an immunological disorder characterised by defective antibody production. Patients with CVID have a high risk of gastric cancer. It has been suggested that gastric cancer results from an interaction between environmental factors and a genetic predisposition. The role of Helicobacter pylori as an environmental factor in gastric carcinogenesis is of current interest. Moreover, p53 gene mutations have been reported in gastric cancer. This study focuses on the gastric pathology of patients with CVID and correlation with H pylori infection. METHODS: Thirty four consecutive dyspeptic patients with CVID (mean age 49.6 years, range 14-72; 17 men) were included in the study. An upper gastrointestinal endoscopy was performed and biopsy specimens were taken from the antrum, incisura angularis, and gastric body. Biopsies were used for histological assessment, to identify the presence of H pylori, and to evaluate p53 overexpression. RESULTS: H pylori infection was detected in 14/34 (41%) patients. Chronic active gastritis involving both antrum and body was observed more frequently in H pylori positive (79%) than H pylori negative (20%) patients (p = 0.001). Similarly, a histological feature of multifocal atrophic gastritis was found more frequently in infected (50%) than uninfected patients (10%) (p = 0.012). In addition, one case of gastric adenocarcinoma and another of notable dysplasia were observed in the H pylori positive group. Overexpression of p53 was found in six (18%) patients, including one with normal gastric mucosa. CONCLUSIONS: It can be hypothesised that both H pylori and p53 alterations play a role in the gastric carcinogenesis of patients with CVID. (+info)Effect of IVIG on the hair regrowth in a common variable immune deficiency patient with alopecia universalis. (4/340)
Common variable immune deficiency (CVID) is associated with a variety of autoimmune diseases. Alopecia universalis (AU), believed to have an autoimmune basis, has been found in 1.6% of patients with CVID. Intravenous immunoglobulin (IVIG) therapy is used in various immunodeficiency disorders including CVID, and benefit has been shown in the therapy of autoimmune diseases. We report a patient with CVID and AU treated with IVIG who experienced significant hair regrowth. An 8-year-old girl with CVID and AU was treated with IVIG 400 mg/kg every 4 weeks. Since her second dose of IVIG, regrowth of eyelashes, eyebrows, body and scalp hair was observed in this patient. At present, about 1 year treat-meant of IVIG, significant hair regrowth is noted with 5-6 cm of scalp hair. We believe that IVIG may be beneficial in the treatment of AU, at least in patients with CVID. (+info)Identification of a subset of common variable immunodeficiency patients with impaired B-cell protein tyrosine phosphorylation. (5/340)
The mechanisms responsible for common variable immunodeficiency syndrome (CVID) are as yet unknown. In the present study, we show that the B-cell dysfunction in a subset of CVID patients is caused by defective protein tyrosine phosphorylation (PTP). We demonstrated that the PTP level and immunoglobulin (Ig) secretion malfunctions can be successfully repaired when normal plasma membrane components are implanted into these patients' B cells. Stimulation of CVID patients' peripheral blood mononucleated cells with anti-Ig antibody revealed that 7 of 11 patients had lower PTP levels than those found in the normal donor cells. Plasma membrane implantation to the cells of these patients resulted in elevated PTP levels which reached normal levels upon stimulation with anti-human Ig antibody. The results revealed two distinct groups of CVID patients. The first group included patients whose B cells expressed low PTP levels after Ig stimulation. In these patients the plasma membrane implantation restored the normal PTP level as well as the ability to secrete IgM and/or IgG after B-cell stimulation. In the second group, patients whose B cells expressed a normal PTP level after Ig stimulation, with no restoration of their ability to secrete Ig upon plasma membrane implantation and lipopolysaccharide stimulation. We conclude that the first group has an early signal transduction defect located in the B-cell plasma membrane, while in the second group the defect is located elsewhere. (+info)Up-regulation of IL-12 in monocytes: a fundamental defect in common variable immunodeficiency. (6/340)
We show that LPS-stimulated circulating CD14-positive monocytes from patients with common variable immunodeficiency (CVID) express a higher proportion of intracellular IL-12-positive cells than monocytes from patients with X-linked agammaglobulinemia or normal subjects. We used four-color flow cytometry and measured IL-12 with an Ab to the p40 subunit following stimulation with LPS. The raised IL-12 is associated with an increased frequency of IFN-gamma-positive T cells, but not of IFN-gamma-positive CD56+ NK cells. These increases in frequency of cytokine-positive cells are due to a decrease in the absolute numbers of circulating monocytes and T cells that are negative for IL-12 and IFN-gamma, respectively. The increased frequency of IL-12-positive monocytes appears to be selective because TNF-alpha was not increased, and is thus unlikely to reflect a general activation. Chronic infection is also unlikely to explain our data since cells from X-linked agammaglobulinemia patients with a similar Ig deficiency do not show these changes. Our data suggest a fundamental abnormality in the IL-12/IFN-gamma circuit in CVID, with up-regulation of IL-12 being the "primary" factor. This imbalance is likely to skew the immune response away from Ab production and also explains the failure of CVID T cells to make Ag-specific memory cells and the chronic inflammatory and granulomatous complications that are a feature of CVID. This disease appears to be a rare example of a polarized Th1-type response and may in part be due to a genetic defect in the control of IL-12 production. (+info)Lymphoid interstitial pneumonitis associated with common variable hypogammaglobulinaemia treated with cyclosporin A. (7/340)
Lymphoid interstitial pneumonitis (LIP) is a rare clinicopathological entity that may be associated with common variable immune deficiency (CVID) and may lead to respiratory failure and death. Some patients may respond to prolonged corticosteroid treatment. We hypothesised that, in view of the predominant T cell nature of LIP, cyclosporin A would be a more appropriate choice of immunosuppressive agent and report the first case of its successful use in a woman with LIP associated with CVID. (+info)Long-term outcome of chronic hepatitis C virus infection in primary hypogammaglobulinaemia. (8/340)
The clinical course of HCV infection in patients with primary hypogammaglobulinaemia appears to be more severe than in immunocompetent patients. We studied the long-term course of chronic HCV infection in 20 Norwegian hypogammaglobulinaemia patients with a 13-15 year known history of HCV infection. Twelve of 20 patients developed cirrhosis during the observation period (1984-1999), and the remaining eight also had chronic liver disease verified by liver biopsy in the majority of the cases. Eleven of the 20 patients are dead. Two died following liver transplantation for HCV cirrhosis. Five died due to terminal liver failure without receiving a liver allograft. Two patients died from other causes, but with advanced liver disease contributing to the outcome, while two deaths were unrelated to the HCV infection. Among patients with common variable immunodeficiency (CVI), five out of six are dead. Two patients cleared the hepatitis C virus 3 years following interferon monotherapy, while three patients achieved a sustained response to combination therapy with interferon and ribavirin. Viral load did not seem to have a major impact on disease progression. Our results emphasize the severity of hepatitis C virus infection in patients with hypogammaglobulinaemia. Patients with CVI appear to have the poorest prognosis. (+info)Common Variable Immunodeficiency (CVID) is a type of primary immunodeficiency disorder characterized by reduced levels of immunoglobulins (also known as antibodies) in the blood, which makes an individual more susceptible to infections. The term "common" refers to its prevalence compared to other types of immunodeficiencies, and "variable" indicates the variability in the severity and types of symptoms among affected individuals.
Immunoglobulins are proteins produced by the immune system to help fight off infections caused by bacteria, viruses, and other pathogens. In CVID, there is a deficiency in the production or function of these immunoglobulins, particularly IgG, IgA, and/or IgM. This results in recurrent infections, chronic inflammation, and an increased risk of developing autoimmune disorders and cancer.
Symptoms of CVID can include:
1. Recurrent sinus, ear, and lung infections
2. Gastrointestinal issues, such as diarrhea, bloating, and malabsorption
3. Autoimmune disorders, like rheumatoid arthritis, lupus, or inflammatory bowel disease
4. Increased risk of certain cancers, particularly lymphomas
5. Fatigue and poor growth in children
6. Delayed puberty in adolescents
7. Lung damage due to recurrent infections
8. Poor response to vaccinations
The exact cause of CVID is not fully understood, but it is believed to be related to genetic factors. In some cases, a family history of immunodeficiency disorders may be present. Diagnosis typically involves blood tests to measure immunoglobulin levels and other immune system components, as well as genetic testing to identify any known genetic mutations associated with CVID. Treatment usually consists of regular infusions of immunoglobulins to replace the missing antibodies and help prevent infections.
Agammaglobulinemia is a medical condition characterized by a severe deficiency or complete absence of gamma globulins (a type of antibodies) in the blood. This deficiency results from a lack of functional B cells, which are a type of white blood cell that produces antibodies to help fight off infections.
There are two main types of agammaglobulinemia: X-linked agammaglobulinemia (XLA) and autosomal recessive agammaglobulinemia (ARA). XLA is caused by mutations in the BTK gene and primarily affects males, while ARA is caused by mutations in other genes and can affect both males and females.
People with agammaglobulinemia are at increased risk for recurrent bacterial infections, particularly respiratory tract infections such as pneumonia and sinusitis. They may also be more susceptible to certain viral and parasitic infections. Treatment typically involves replacement therapy with intravenous immunoglobulin (IVIG) to provide the patient with functional antibodies.
IgA deficiency is a condition characterized by significantly reduced levels or absence of secretory immunoglobulin A (IgA), an important antibody that plays a crucial role in the immune function of mucous membranes lining the respiratory and gastrointestinal tracts. IgA helps to prevent the attachment and multiplication of pathogens, such as bacteria and viruses, on these surfaces.
In individuals with IgA deficiency, the lack of adequate IgA levels makes them more susceptible to recurrent infections, allergies, and autoimmune disorders. The condition can be asymptomatic or may present with various symptoms, such as respiratory tract infections, gastrointestinal issues, and chronic sinusitis. IgA deficiency is typically diagnosed through blood tests that measure the immunoglobulin levels. While there is no cure for IgA deficiency, treatment focuses on managing symptoms and preventing infections through medications, immunizations, and lifestyle modifications.
IgG deficiency is a type of immunodeficiency disorder characterized by reduced levels of immunoglobulin G (IgG) antibodies in the blood. IgG is the most common type of antibody in our body and plays a crucial role in fighting against infections.
There are four subclasses of IgG (IgG1, IgG2, IgG3, and IgG4), and a deficiency in one or more of these subclasses can lead to recurrent infections, particularly of the respiratory tract, such as sinusitis, bronchitis, and pneumonia. People with IgG deficiency may also be more susceptible to autoimmune diseases and allergies.
IgG deficiency can be inherited or acquired, and it is usually diagnosed through blood tests that measure the levels of IgG and other immunoglobulins in the blood. Treatment typically involves preventing infections through vaccinations, antibiotics to treat infections, and in some cases, replacement therapy with intravenous immunoglobulin (IVIG) to boost the immune system.
Immunologic deficiency syndromes refer to a group of disorders characterized by defective functioning of the immune system, leading to increased susceptibility to infections and malignancies. These deficiencies can be primary (genetic or congenital) or secondary (acquired due to environmental factors, medications, or diseases).
Primary immunodeficiency syndromes (PIDS) are caused by inherited genetic mutations that affect the development and function of immune cells, such as T cells, B cells, and phagocytes. Examples include severe combined immunodeficiency (SCID), common variable immunodeficiency (CVID), Wiskott-Aldrich syndrome, and X-linked agammaglobulinemia.
Secondary immunodeficiency syndromes can result from various factors, including:
1. HIV/AIDS: Human Immunodeficiency Virus infection leads to the depletion of CD4+ T cells, causing profound immune dysfunction and increased vulnerability to opportunistic infections and malignancies.
2. Medications: Certain medications, such as chemotherapy, immunosuppressive drugs, and long-term corticosteroid use, can impair immune function and increase infection risk.
3. Malnutrition: Deficiencies in essential nutrients like protein, vitamins, and minerals can weaken the immune system and make individuals more susceptible to infections.
4. Aging: The immune system naturally declines with age, leading to an increased incidence of infections and poorer vaccine responses in older adults.
5. Other medical conditions: Chronic diseases such as diabetes, cancer, and chronic kidney or liver disease can also compromise the immune system and contribute to immunodeficiency syndromes.
Immunologic deficiency syndromes require appropriate diagnosis and management strategies, which may include antimicrobial therapy, immunoglobulin replacement, hematopoietic stem cell transplantation, or targeted treatments for the underlying cause.
Intravenous Immunoglobulins (IVIG) are a preparation of antibodies, specifically immunoglobulins, that are derived from the plasma of healthy donors. They are administered intravenously to provide passive immunity and help boost the immune system's response in individuals with weakened or compromised immune systems. IVIG can be used for various medical conditions such as primary immunodeficiency disorders, secondary immunodeficiencies, autoimmune diseases, and some infectious diseases. The administration of IVIG can help prevent infections, reduce the severity and frequency of infections, and manage the symptoms of certain autoimmune disorders. It is important to note that while IVIG provides temporary immunity, it does not replace a person's own immune system.
Dysgammaglobulinemia is a medical term that refers to an abnormal gamma globulin or immunoglobulin (antibody) level in the blood. Gamma globulins are proteins that play a crucial role in the immune system and help fight off infections. Immunoglobulins are classified into five types (IgA, IgD, IgE, IgG, and IgM), each with a specific function in the immune response.
In dysgammaglobulinemia, there is an imbalance in the levels of these immunoglobulins, which can be either elevated or decreased. This condition can result from various underlying causes, including genetic disorders, autoimmune diseases, infections, and malignancies that affect the bone marrow or lymphatic system.
Depending on the specific pattern of immunoglobulin levels, dysgammaglobulinemia can be further classified into different types, such as:
1. Hypogammaglobulinemia - a decrease in one or more classes of immunoglobulins
2. Agammaglobulinemia - a severe deficiency or absence of all classes of immunoglobulins
3. Hypergammaglobulinemia - an elevation of one or more classes of immunoglobulins
Dysgammaglobulinemia can lead to increased susceptibility to infections, autoimmune disorders, and other health complications. Therefore, it is essential to identify the underlying cause and provide appropriate treatment to manage the condition and prevent further complications.
The Transmembrane Activator and CAML Interactor protein (also known as TACI or TNFRSF13B) is a type I transmembrane protein that belongs to the tumor necrosis factor receptor superfamily. It is involved in the regulation of immune responses, specifically in the activation and differentiation of B cells, which are a type of white blood cell that plays a central role in the humoral immune response.
TACI has two main ligands, or binding partners: A Proliferation-Inducing Ligand (APRIL) and B cell Activating Factor (BAFF). These ligands bind to TACI and activate downstream signaling pathways that lead to the activation and differentiation of B cells.
Mutations in the TACI gene have been associated with various immune disorders, including common variable immunodeficiency (CVID), a primary immunodeficiency disorder characterized by low levels of antibodies and recurrent infections. Additionally, variations in the TACI gene have been linked to an increased risk of developing autoimmune diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA).
Bronchiectasis is a medical condition characterized by permanent, abnormal widening and thickening of the walls of the bronchi (the airways leading to the lungs). This can lead to recurrent respiratory infections, coughing, and the production of large amounts of sputum. The damage to the airways is usually irreversible and can be caused by various factors such as bacterial or viral infections, genetic disorders, immune deficiencies, or exposure to environmental pollutants. In some cases, the cause may remain unknown. Treatment typically includes chest physiotherapy, bronchodilators, antibiotics, and sometimes surgery.
B-lymphocytes, also known as B-cells, are a type of white blood cell that plays a key role in the immune system's response to infection. They are responsible for producing antibodies, which are proteins that help to neutralize or destroy pathogens such as bacteria and viruses.
When a B-lymphocyte encounters a pathogen, it becomes activated and begins to divide and differentiate into plasma cells, which produce and secrete large amounts of antibodies specific to the antigens on the surface of the pathogen. These antibodies bind to the pathogen, marking it for destruction by other immune cells such as neutrophils and macrophages.
B-lymphocytes also have a role in presenting antigens to T-lymphocytes, another type of white blood cell involved in the immune response. This helps to stimulate the activation and proliferation of T-lymphocytes, which can then go on to destroy infected cells or help to coordinate the overall immune response.
Overall, B-lymphocytes are an essential part of the adaptive immune system, providing long-lasting immunity to previously encountered pathogens and helping to protect against future infections.
Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by the immune system's B cells in response to the presence of foreign substances, such as bacteria, viruses, and toxins. These Y-shaped proteins play a crucial role in identifying and neutralizing pathogens and other antigens, thereby protecting the body against infection and disease.
Immunoglobulins are composed of four polypeptide chains: two identical heavy chains and two identical light chains, held together by disulfide bonds. The variable regions of these chains form the antigen-binding sites, which recognize and bind to specific epitopes on antigens. Based on their heavy chain type, immunoglobulins are classified into five main isotypes or classes: IgA, IgD, IgE, IgG, and IgM. Each class has distinct functions in the immune response, such as providing protection in different body fluids and tissues, mediating hypersensitivity reactions, and aiding in the development of immunological memory.
In medical settings, immunoglobulins can be administered therapeutically to provide passive immunity against certain diseases or to treat immune deficiencies, autoimmune disorders, and other conditions that may benefit from immunomodulation.
B-lymphocytes, also known as B-cells, are a type of white blood cell that plays a central role in the humoral immune response. They are responsible for producing antibodies, which are proteins that help to neutralize or destroy pathogens such as viruses and bacteria.
B-lymphocyte subsets refer to distinct populations of B-cells that can be identified based on their surface receptors and functional characteristics. Some common B-lymphocyte subsets include:
1. Naive B-cells: These are mature B-cells that have not yet been exposed to an antigen. They express surface receptors called immunoglobulin M (IgM) and immunoglobulin D (IgD).
2. Memory B-cells: These are B-cells that have previously encountered an antigen and mounted an immune response. They express high levels of surface immunoglobulins and can quickly differentiate into antibody-secreting plasma cells upon re-exposure to the same antigen.
3. Plasma cells: These are fully differentiated B-cells that secrete large amounts of antibodies in response to an antigen. They lack surface immunoglobulins and do not undergo further division.
4. Regulatory B-cells: These are a subset of B-cells that modulate the immune response by producing anti-inflammatory cytokines and suppressing the activation of other immune cells.
5. B-1 cells: These are a population of B-cells that are primarily found in the peripheral blood and mucosal tissues. They produce natural antibodies that provide early protection against pathogens and help to maintain tissue homeostasis.
Understanding the different B-lymphocyte subsets and their functions is important for diagnosing and treating immune-related disorders, including autoimmune diseases, infections, and cancer.
Splenomegaly is a medical term that refers to an enlargement or expansion of the spleen beyond its normal size. The spleen is a vital organ located in the upper left quadrant of the abdomen, behind the stomach and below the diaphragm. It plays a crucial role in filtering the blood, fighting infections, and storing red and white blood cells and platelets.
Splenomegaly can occur due to various underlying medical conditions, including infections, liver diseases, blood disorders, cancer, and inflammatory diseases. The enlarged spleen may put pressure on surrounding organs, causing discomfort or pain in the abdomen, and it may also lead to a decrease in red and white blood cells and platelets, increasing the risk of anemia, infections, and bleeding.
The diagnosis of splenomegaly typically involves a physical examination, medical history, and imaging tests such as ultrasound, CT scan, or MRI. Treatment depends on the underlying cause and may include medications, surgery, or other interventions to manage the underlying condition.
Aortic arch syndromes are a group of conditions that affect the aortic arch, which is the curved portion of the aorta that arises from the left ventricle of the heart and gives rise to the major branches of the arterial system. These syndromes are typically caused by congenital abnormalities or degenerative changes in the aorta and can result in various complications, such as obstruction of blood flow, aneurysm formation, and dissection.
There are several types of aortic arch syndromes, including:
1. Coarctation of the Aorta: This is a narrowing of the aorta at the point where it leaves the heart, just distal to the origin of the left subclavian artery. It can cause hypertension in the upper extremities and reduced blood flow to the lower extremities.
2. Aortic Arch Aneurysm: This is a localized dilation or bulging of the aorta in the region of the aortic arch. It can lead to dissection, rupture, or embolism.
3. Aortic Arch Dissection: This is a separation of the layers of the aortic wall, which can result from hypertension, trauma, or genetic disorders such as Marfan syndrome. It can cause severe chest pain, shortness of breath, and shock.
4. Kommerell's Diverticulum: This is an outpouching or bulge in the aorta at the origin of the ligamentum arteriosum, which is a remnant of the ductus arteriosus. It can cause compression of the airways or esophagus and increase the risk of dissection or rupture.
5. Abernethy Malformation: This is a rare congenital anomaly in which there is an abnormal connection between the portal vein and systemic venous circulation, leading to the bypass of the liver. It can cause various complications such as hepatic encephalopathy, pulmonary hypertension, and liver tumors.
The diagnosis and management of aortic arch syndromes require a multidisciplinary approach involving cardiologists, radiologists, surgeons, and other specialists. Treatment options may include medications, endovascular procedures, or surgical interventions depending on the severity and location of the lesion.
Immunoglobulin M (IgM) is a type of antibody that is primarily found in the blood and lymph fluid. It is the first antibody to be produced in response to an initial exposure to an antigen, making it an important part of the body's primary immune response. IgM antibodies are large molecules that are composed of five basic units, giving them a pentameric structure. They are primarily found on the surface of B cells as membrane-bound immunoglobulins (mlgM), where they function as receptors for antigens. Once an mlgM receptor binds to an antigen, it triggers the activation and differentiation of the B cell into a plasma cell that produces and secretes large amounts of soluble IgM antibodies.
IgM antibodies are particularly effective at agglutination (clumping) and complement activation, which makes them important in the early stages of an immune response to help clear pathogens from the bloodstream. However, they are not as stable or long-lived as other types of antibodies, such as IgG, and their levels tend to decline after the initial immune response has occurred.
In summary, Immunoglobulin M (IgM) is a type of antibody that plays a crucial role in the primary immune response to antigens by agglutination and complement activation. It is primarily found in the blood and lymph fluid, and it is produced by B cells after they are activated by an antigen.
Pseudolymphoma is a term used to describe a benign reactive lymphoid hyperplasia that mimics the clinical and histopathological features of malignant lymphomas. It is also known as pseudolymphomatous cutis or reactive lymphoid hyperplasia.
Pseudolymphoma can occur in various organs, but it is most commonly found in the skin. It is usually caused by a localized immune response to an antigenic stimulus such as insect bites, tattoos, radiation therapy, or certain medications. The condition presents as a solitary or multiple nodular lesions that may resemble lymphoma both clinically and histologically.
Histologically, pseudolymphoma is characterized by a dense infiltrate of lymphocytes, plasma cells, and other immune cells, which can mimic the appearance of malignant lymphoma. However, unlike malignant lymphomas, pseudolymphomas lack cytological atypia, mitotic activity, and clonal proliferation of lymphoid cells.
Pseudolymphoma is usually a self-limiting condition that resolves spontaneously or with the removal of the antigenic stimulus. However, in some cases, it may persist or recur, requiring further evaluation and treatment to exclude malignant lymphoma.
T-lymphocytopenia, idiopathic CD4-positive, also known as Idiopathic CD4 Lymphopenia (ICL), is a rare medical condition characterized by a significant decrease in the number of CD4+ T lymphocytes in the peripheral blood without an identifiable cause. CD4+ T cells are crucial for immune function and protection against certain types of infections, particularly those caused by viruses such as HIV.
ICL is typically defined as a CD4+ T-lymphocyte count below 300 cells/μL in the absence of HIV infection or any other known immunodeficiency disorder. The exact cause of ICL remains unknown, although it has been associated with genetic factors and autoimmune disorders.
People with ICL may be at increased risk for certain types of infections, such as opportunistic infections, which can occur when the immune system is weakened. However, the severity and frequency of infections in individuals with ICL are generally less than those seen in people with HIV-associated CD4 lymphopenia.
Regular monitoring of CD4+ T-lymphocyte counts and appropriate management of any infections that occur are important for people with ICL to maintain their overall health and well-being.
Lymphocyte activation is the process by which B-cells and T-cells (types of lymphocytes) become activated to perform effector functions in an immune response. This process involves the recognition of specific antigens presented on the surface of antigen-presenting cells, such as dendritic cells or macrophages.
The activation of B-cells leads to their differentiation into plasma cells that produce antibodies, while the activation of T-cells results in the production of cytotoxic T-cells (CD8+ T-cells) that can directly kill infected cells or helper T-cells (CD4+ T-cells) that assist other immune cells.
Lymphocyte activation involves a series of intracellular signaling events, including the binding of co-stimulatory molecules and the release of cytokines, which ultimately result in the expression of genes involved in cell proliferation, differentiation, and effector functions. The activation process is tightly regulated to prevent excessive or inappropriate immune responses that can lead to autoimmunity or chronic inflammation.
Complement C4a is a protein fragment or cleavage product generated during the activation of the complement system, which is a part of the immune system. The complement system helps to eliminate pathogens and damaged cells by marking them for destruction and direct lysis. Complement component 4 (C4) is one of the key proteins in this cascade, and it gets cleaved into C4a and C4b during the activation process.
C4a is a small anaphylatoxin with a molecular weight of approximately 9 kDa. It has chemotactic properties, meaning it can attract immune cells like neutrophils to the site of complement activation. Additionally, C4a can induce histamine release from mast cells and basophils, contributing to local inflammation. However, its precise physiological role in the immune response is not entirely clear, and dysregulation of C4a production has been implicated in several pathological conditions, such as autoimmune diseases and allergies.
Immunoglobulin class switching, also known as isotype switching or class switch recombination (CSR), is a biological process that occurs in B lymphocytes as part of the adaptive immune response. This mechanism allows a mature B cell to change the type of antibody it produces from one class to another (e.g., from IgM to IgG, IgA, or IgE) while keeping the same antigen-binding specificity.
During immunoglobulin class switching, the constant region genes of the heavy chain undergo a DNA recombination event, which results in the deletion of the original constant region exons and the addition of new constant region exons downstream. This switch allows the B cell to express different effector functions through the production of antibodies with distinct constant regions, tailoring the immune response to eliminate pathogens more effectively. The process is regulated by various cytokines and signals from T cells and is critical for mounting an effective humoral immune response.
A granuloma is a small, nodular inflammatory lesion that occurs in various tissues in response to chronic infection, foreign body reaction, or autoimmune conditions. Histologically, it is characterized by the presence of epithelioid macrophages, which are specialized immune cells with enlarged nuclei and abundant cytoplasm, often arranged in a palisading pattern around a central area containing necrotic debris, microorganisms, or foreign material.
Granulomas can be found in various medical conditions such as tuberculosis, sarcoidosis, fungal infections, and certain autoimmune disorders like Crohn's disease. The formation of granulomas is a complex process involving both innate and adaptive immune responses, which aim to contain and eliminate the offending agent while minimizing tissue damage.
Immunoglobulin G (IgG) is a type of antibody, which is a protective protein produced by the immune system in response to foreign substances like bacteria or viruses. IgG is the most abundant type of antibody in human blood, making up about 75-80% of all antibodies. It is found in all body fluids and plays a crucial role in fighting infections caused by bacteria, viruses, and toxins.
IgG has several important functions:
1. Neutralization: IgG can bind to the surface of bacteria or viruses, preventing them from attaching to and infecting human cells.
2. Opsonization: IgG coats the surface of pathogens, making them more recognizable and easier for immune cells like neutrophils and macrophages to phagocytose (engulf and destroy) them.
3. Complement activation: IgG can activate the complement system, a group of proteins that work together to help eliminate pathogens from the body. Activation of the complement system leads to the formation of the membrane attack complex, which creates holes in the cell membranes of bacteria, leading to their lysis (destruction).
4. Antibody-dependent cellular cytotoxicity (ADCC): IgG can bind to immune cells like natural killer (NK) cells and trigger them to release substances that cause target cells (such as virus-infected or cancerous cells) to undergo apoptosis (programmed cell death).
5. Immune complex formation: IgG can form immune complexes with antigens, which can then be removed from the body through various mechanisms, such as phagocytosis by immune cells or excretion in urine.
IgG is a critical component of adaptive immunity and provides long-lasting protection against reinfection with many pathogens. It has four subclasses (IgG1, IgG2, IgG3, and IgG4) that differ in their structure, function, and distribution in the body.
Tumor Necrosis Factor Ligand Superfamily Member 13 (TNFSF13), also known as APRIL (A Proliferation-Inducing Ligand), is a type II transmembrane protein and a member of the tumor necrosis factor (TNF) ligand superfamily. It plays a crucial role in the immune system, particularly in the activation, proliferation, and differentiation of B cells, which are key players in the humoral immune response.
TNFSF13 is expressed by various cell types, including macrophages, dendritic cells, and neutrophils. It binds to two receptors: TACI (Transmembrane Activator and Calcium Modulator and Cyclophilin Ligand Interactor) and BCMA (B-cell Maturation Antigen), which are primarily found on the surface of B cells. The interaction between TNFSF13 and its receptors promotes the survival, proliferation, and differentiation of B cells into plasma cells, ultimately leading to increased antibody production.
Dysregulation of TNFSF13 has been implicated in several autoimmune and inflammatory diseases, such as rheumatoid arthritis, systemic lupus erythematosus (SLE), and multiple sclerosis (MS). Therefore, targeting this molecule or its signaling pathways has been a focus of research for the development of novel therapeutic strategies in these conditions.
T-lymphocytes, also known as T-cells, are a type of white blood cell that plays a key role in the adaptive immune system's response to infection. They are produced in the bone marrow and mature in the thymus gland. There are several different types of T-cells, including CD4+ helper T-cells, CD8+ cytotoxic T-cells, and regulatory T-cells (Tregs).
CD4+ helper T-cells assist in activating other immune cells, such as B-lymphocytes and macrophages. They also produce cytokines, which are signaling molecules that help coordinate the immune response. CD8+ cytotoxic T-cells directly kill infected cells by releasing toxic substances. Regulatory T-cells help maintain immune tolerance and prevent autoimmune diseases by suppressing the activity of other immune cells.
T-lymphocytes are important in the immune response to viral infections, cancer, and other diseases. Dysfunction or depletion of T-cells can lead to immunodeficiency and increased susceptibility to infections. On the other hand, an overactive T-cell response can contribute to autoimmune diseases and chronic inflammation.
Immunophenotyping is a medical laboratory technique used to identify and classify cells, usually in the context of hematologic (blood) disorders and malignancies (cancers), based on their surface or intracellular expression of various proteins and antigens. This technique utilizes specific antibodies tagged with fluorochromes, which bind to the target antigens on the cell surface or within the cells. The labeled cells are then analyzed using flow cytometry, allowing for the detection and quantification of multiple antigenic markers simultaneously.
Immunophenotyping helps in understanding the distribution of different cell types, their subsets, and activation status, which can be crucial in diagnosing various hematological disorders, immunodeficiencies, and distinguishing between different types of leukemias, lymphomas, and other malignancies. Additionally, it can also be used to monitor the progression of diseases, evaluate the effectiveness of treatments, and detect minimal residual disease (MRD) during follow-up care.
Immunoglobulin A (IgA) is a type of antibody that plays a crucial role in the immune function of the human body. It is primarily found in external secretions, such as saliva, tears, breast milk, and sweat, as well as in mucous membranes lining the respiratory and gastrointestinal tracts. IgA exists in two forms: a monomeric form found in serum and a polymeric form found in secretions.
The primary function of IgA is to provide immune protection at mucosal surfaces, which are exposed to various environmental antigens, such as bacteria, viruses, parasites, and allergens. By doing so, it helps prevent the entry and colonization of pathogens into the body, reducing the risk of infections and inflammation.
IgA functions by binding to antigens present on the surface of pathogens or allergens, forming immune complexes that can neutralize their activity. These complexes are then transported across the epithelial cells lining mucosal surfaces and released into the lumen, where they prevent the adherence and invasion of pathogens.
In summary, Immunoglobulin A (IgA) is a vital antibody that provides immune defense at mucosal surfaces by neutralizing and preventing the entry of harmful antigens into the body.
Opportunistic infections (OIs) are infections that occur more frequently or are more severe in individuals with weakened immune systems, often due to a underlying condition such as HIV/AIDS, cancer, or organ transplantation. These infections are caused by microorganisms that do not normally cause disease in people with healthy immune function, but can take advantage of an opportunity to infect and cause damage when the body's defense mechanisms are compromised. Examples of opportunistic infections include Pneumocystis pneumonia, tuberculosis, candidiasis (thrush), and cytomegalovirus infection. Preventive measures, such as antimicrobial medications and vaccinations, play a crucial role in reducing the risk of opportunistic infections in individuals with weakened immune systems.
HIV-1 (Human Immunodeficiency Virus type 1) is a species of the retrovirus genus that causes acquired immunodeficiency syndrome (AIDS). It is primarily transmitted through sexual contact, exposure to infected blood or blood products, and from mother to child during pregnancy, childbirth, or breastfeeding. HIV-1 infects vital cells in the human immune system, such as CD4+ T cells, macrophages, and dendritic cells, leading to a decline in their numbers and weakening of the immune response over time. This results in the individual becoming susceptible to various opportunistic infections and cancers that ultimately cause death if left untreated. HIV-1 is the most prevalent form of HIV worldwide and has been identified as the causative agent of the global AIDS pandemic.
CD27 is a protein that is found on the surface of certain immune cells, including T cells and B cells. It is a type of molecule known as a cell-surface antigen, which can be recognized by other immune cells and used to target those cells for activation or destruction. CD27 plays a role in the regulation of the immune response, particularly in the activation and differentiation of T cells.
CD27 is also a member of the tumor necrosis factor receptor (TNFR) superfamily, which means that it has a specific structure and function that allows it to interact with other molecules called ligands. The interaction between CD27 and its ligand, CD70, helps to activate T cells and promote their survival and proliferation.
In addition to its role in the immune response, CD27 has also been studied as a potential target for cancer immunotherapy. Because CD27 is expressed on certain types of tumor cells, it may be possible to use therapies that target CD27 to stimulate an immune response against the tumor and help to destroy it. However, more research is needed to determine the safety and effectiveness of these approaches.
Immunologic memory, also known as adaptive immunity, refers to the ability of the immune system to recognize and mount a more rapid and effective response upon subsequent exposure to a pathogen or antigen that it has encountered before. This is a key feature of the vertebrate immune system and allows for long-term protection against infectious diseases.
Immunologic memory is mediated by specialized cells called memory T cells and B cells, which are produced during the initial response to an infection or immunization. These cells persist in the body after the pathogen has been cleared and can quickly respond to future encounters with the same or similar antigens. This rapid response leads to a more effective and efficient elimination of the pathogen, resulting in fewer symptoms and reduced severity of disease.
Immunologic memory is the basis for vaccines, which work by exposing the immune system to a harmless form of a pathogen or its components, inducing an initial response and generating memory cells that provide long-term protection against future infections.
Cardiovascular abnormalities refer to structural or functional anomalies in the heart or blood vessels. These abnormalities can be present at birth (congenital) or acquired later in life. They can affect the heart's chambers, valves, walls, or blood vessels, leading to various complications such as heart failure, stroke, or even death if left untreated.
Examples of congenital cardiovascular abnormalities include:
1. Septal defects - holes in the walls separating the heart's chambers (atrial septal defect, ventricular septal defect)
2. Valvular stenosis or insufficiency - narrowing or leakage of the heart valves
3. Patent ductus arteriosus - a persistent opening between the aorta and pulmonary artery
4. Coarctation of the aorta - narrowing of the aorta
5. Tetralogy of Fallot - a combination of four heart defects, including ventricular septal defect, overriding aorta, pulmonary stenosis, and right ventricular hypertrophy
Examples of acquired cardiovascular abnormalities include:
1. Atherosclerosis - the buildup of plaque in the arteries, leading to narrowing or blockage
2. Cardiomyopathy - disease of the heart muscle, causing it to become enlarged, thickened, or stiffened
3. Hypertension - high blood pressure, which can damage the heart and blood vessels over time
4. Myocardial infarction (heart attack) - damage to the heart muscle due to blocked blood supply
5. Infective endocarditis - infection of the inner lining of the heart chambers and valves
These abnormalities can be diagnosed through various tests, such as echocardiography, electrocardiogram (ECG), stress testing, cardiac catheterization, or magnetic resonance imaging (MRI). Treatment options depend on the type and severity of the abnormality and may include medications, medical procedures, or surgery.
Interleukin-2 (IL-2) is a type of cytokine, which are signaling molecules that mediate and regulate immunity, inflammation, and hematopoiesis. Specifically, IL-2 is a growth factor for T cells, a type of white blood cell that plays a central role in the immune response. It is primarily produced by CD4+ T cells (also known as T helper cells) and stimulates the proliferation and differentiation of activated T cells, including effector T cells and regulatory T cells. IL-2 also has roles in the activation and function of other immune cells, such as B cells, natural killer cells, and dendritic cells. Dysregulation of IL-2 production or signaling can contribute to various pathological conditions, including autoimmune diseases, chronic infections, and cancer.
A lymphocyte count is a laboratory test that measures the number of white blood cells called lymphocytes in a sample of blood. Lymphocytes are a vital part of the immune system and help fight off infections and diseases. A normal lymphocyte count ranges from 1,000 to 4,800 cells per microliter (µL) of blood for adults.
An abnormal lymphocyte count can indicate an infection, immune disorder, or blood cancer. A low lymphocyte count is called lymphopenia, while a high lymphocyte count is called lymphocytosis. The cause of an abnormal lymphocyte count should be investigated through further testing and clinical evaluation.
Hemolytic anemia, autoimmune is a type of anemia characterized by the premature destruction of red blood cells (RBCs) in which the immune system mistakenly attacks and destroys its own RBCs. This occurs when the body produces autoantibodies that bind to the surface of RBCs, leading to their rupture (hemolysis). The symptoms may include fatigue, weakness, shortness of breath, and dark colored urine. The diagnosis is made through blood tests that measure the number and size of RBCs, reticulocyte count, and the presence of autoantibodies. Treatment typically involves suppressing the immune system with medications such as corticosteroids or immunosuppressive drugs, and sometimes removal of the spleen (splenectomy) may be necessary.
Interstitial lung diseases (ILDs) are a group of disorders characterized by inflammation and scarring (fibrosis) in the interstitium, the tissue and space around the air sacs (alveoli) of the lungs. The interstitium is where the blood vessels that deliver oxygen to the lungs are located. ILDs can be caused by a variety of factors, including environmental exposures, medications, connective tissue diseases, and autoimmune disorders.
The scarring and inflammation in ILDs can make it difficult for the lungs to expand and contract normally, leading to symptoms such as shortness of breath, cough, and fatigue. The scarring can also make it harder for oxygen to move from the air sacs into the bloodstream.
There are many different types of ILDs, including:
* Idiopathic pulmonary fibrosis (IPF): a type of ILD that is caused by unknown factors and tends to progress rapidly
* Hypersensitivity pneumonitis: an ILD that is caused by an allergic reaction to inhaled substances, such as mold or bird droppings
* Connective tissue diseases: ILDs can be a complication of conditions such as rheumatoid arthritis and scleroderma
* Sarcoidosis: an inflammatory disorder that can affect multiple organs, including the lungs
* Asbestosis: an ILD caused by exposure to asbestos fibers
Treatment for ILDs depends on the specific type of disease and its underlying cause. Some treatments may include corticosteroids, immunosuppressive medications, and oxygen therapy. In some cases, a lung transplant may be necessary.
Acquired Immunodeficiency Syndrome (AIDS) is a chronic, life-threatening condition caused by the Human Immunodeficiency Virus (HIV). AIDS is the most advanced stage of HIV infection, characterized by the significant weakening of the immune system, making the person more susceptible to various opportunistic infections and cancers.
The medical definition of AIDS includes specific criteria based on CD4+ T-cell count or the presence of certain opportunistic infections and diseases. According to the Centers for Disease Control and Prevention (CDC), a person with HIV is diagnosed with AIDS when:
1. The CD4+ T-cell count falls below 200 cells per cubic millimeter of blood (mm3) - a normal range is typically between 500 and 1,600 cells/mm3.
2. They develop one or more opportunistic infections or cancers that are indicative of advanced HIV disease, regardless of their CD4+ T-cell count.
Some examples of these opportunistic infections and cancers include:
* Pneumocystis pneumonia (PCP)
* Candidiasis (thrush) affecting the esophagus, trachea, or lungs
* Cryptococcal meningitis
* Toxoplasmosis of the brain
* Cytomegalovirus disease
* Kaposi's sarcoma
* Non-Hodgkin's lymphoma
* Invasive cervical cancer
It is important to note that with appropriate antiretroviral therapy (ART), people living with HIV can maintain their CD4+ T-cell counts, suppress viral replication, and prevent the progression to AIDS. Early diagnosis and consistent treatment are crucial for managing HIV and improving life expectancy and quality of life.
Simian Immunodeficiency Virus (SIV) is a retrovirus that primarily infects African non-human primates and is the direct ancestor of Human Immunodeficiency Virus type 2 (HIV-2). It is similar to HIV in its structure, replication strategy, and ability to cause an immunodeficiency disease in its host. SIV infection in its natural hosts is typically asymptomatic and non-lethal, but it can cause AIDS-like symptoms in other primate species. Research on SIV in its natural hosts has provided valuable insights into the mechanisms of HIV pathogenesis and potential strategies for prevention and treatment of AIDS.
The CD4-CD8 ratio is a measurement of the relative numbers of two types of immune cells, CD4+ T cells (also known as helper T cells) and CD8+ T cells (also known as cytotoxic T cells), in the blood. The CD4-CD8 ratio is commonly used as a marker of immune function and health.
CD4+ T cells play an important role in the immune response by helping to coordinate the activity of other immune cells, producing chemical signals that activate them, and producing antibodies. CD8+ T cells are responsible for directly killing infected cells and tumor cells.
A normal CD4-CD8 ratio is typically between 1.0 and 3.0. A lower ratio may indicate an impaired immune system, such as in cases of HIV infection or other immunodeficiency disorders. A higher ratio may be seen in some viral infections, autoimmune diseases, or cancer. It's important to note that the CD4-CD8 ratio should be interpreted in conjunction with other laboratory and clinical findings for a more accurate assessment of immune function.
Autoimmunity is a medical condition in which the body's immune system mistakenly attacks and destroys healthy tissues within the body. In normal function, the immune system recognizes and fights off foreign substances such as bacteria, viruses, and toxins. However, when autoimmunity occurs, the immune system identifies self-molecules or tissues as foreign and produces an immune response against them.
This misguided response can lead to chronic inflammation, tissue damage, and impaired organ function. Autoimmune diseases can affect various parts of the body, including the joints, skin, glands, muscles, and blood vessels. Some common examples of autoimmune diseases are rheumatoid arthritis, lupus, multiple sclerosis, type 1 diabetes, Hashimoto's thyroiditis, and Graves' disease.
The exact cause of autoimmunity is not fully understood, but it is believed to involve a combination of genetic, environmental, and lifestyle factors that trigger an abnormal immune response in susceptible individuals. Treatment for autoimmune diseases typically involves managing symptoms, reducing inflammation, and suppressing the immune system's overactive response using medications such as corticosteroids, immunosuppressants, and biologics.
A splenectomy is a surgical procedure in which the spleen is removed from the body. The spleen is an organ located in the upper left quadrant of the abdomen, near the stomach and behind the ribs. It plays several important roles in the body, including fighting certain types of infections, removing old or damaged red blood cells from the circulation, and storing platelets and white blood cells.
There are several reasons why a splenectomy may be necessary, including:
* Trauma to the spleen that cannot be repaired
* Certain types of cancer, such as Hodgkin's lymphoma or non-Hodgkin's lymphoma
* Sickle cell disease, which can cause the spleen to enlarge and become damaged
* A ruptured spleen, which can be life-threatening if not treated promptly
* Certain blood disorders, such as idiopathic thrombocytopenic purpura (ITP) or hemolytic anemia
A splenectomy is typically performed under general anesthesia and may be done using open surgery or laparoscopically. After the spleen is removed, the incision(s) are closed with sutures or staples. Recovery time varies depending on the individual and the type of surgery performed, but most people are able to return to their normal activities within a few weeks.
It's important to note that following a splenectomy, individuals may be at increased risk for certain types of infections, so it's recommended that they receive vaccinations to help protect against these infections. They should also seek medical attention promptly if they develop fever, chills, or other signs of infection.
CD4-positive T-lymphocytes, also known as CD4+ T cells or helper T cells, are a type of white blood cell that plays a crucial role in the immune response. They express the CD4 receptor on their surface and help coordinate the immune system's response to infectious agents such as viruses and bacteria.
CD4+ T cells recognize and bind to specific antigens presented by antigen-presenting cells, such as dendritic cells or macrophages. Once activated, they can differentiate into various subsets of effector cells, including Th1, Th2, Th17, and Treg cells, each with distinct functions in the immune response.
CD4+ T cells are particularly important in the immune response to HIV (human immunodeficiency virus), which targets and destroys these cells, leading to a weakened immune system and increased susceptibility to opportunistic infections. The number of CD4+ T cells is often used as a marker of disease progression in HIV infection, with lower counts indicating more advanced disease.
Infection is defined medically as the invasion and multiplication of pathogenic microorganisms such as bacteria, viruses, fungi, or parasites within the body, which can lead to tissue damage, illness, and disease. This process often triggers an immune response from the host's body in an attempt to eliminate the infectious agents and restore homeostasis. Infections can be transmitted through various routes, including airborne particles, direct contact with contaminated surfaces or bodily fluids, sexual contact, or vector-borne transmission. The severity of an infection may range from mild and self-limiting to severe and life-threatening, depending on factors such as the type and quantity of pathogen, the host's immune status, and any underlying health conditions.
Antibody formation, also known as humoral immune response, is the process by which the immune system produces proteins called antibodies in response to the presence of a foreign substance (antigen) in the body. This process involves several steps:
1. Recognition: The antigen is recognized and bound by a type of white blood cell called a B lymphocyte or B cell, which then becomes activated.
2. Differentiation: The activated B cell undergoes differentiation to become a plasma cell, which is a type of cell that produces and secretes large amounts of antibodies.
3. Antibody production: The plasma cells produce and release antibodies, which are proteins made up of four polypeptide chains (two heavy chains and two light chains) arranged in a Y-shape. Each antibody has two binding sites that can recognize and bind to specific regions on the antigen called epitopes.
4. Neutralization or elimination: The antibodies bind to the antigens, neutralizing them or marking them for destruction by other immune cells. This helps to prevent the spread of infection and protect the body from harmful substances.
Antibody formation is an important part of the adaptive immune response, which allows the body to specifically recognize and respond to a wide variety of pathogens and foreign substances.
CD (cluster of differentiation) antigens are cell-surface proteins that are expressed on leukocytes (white blood cells) and can be used to identify and distinguish different subsets of these cells. They are important markers in the field of immunology and hematology, and are commonly used to diagnose and monitor various diseases, including cancer, autoimmune disorders, and infectious diseases.
CD antigens are designated by numbers, such as CD4, CD8, CD19, etc., which refer to specific proteins found on the surface of different types of leukocytes. For example, CD4 is a protein found on the surface of helper T cells, while CD8 is found on cytotoxic T cells.
CD antigens can be used as targets for immunotherapy, such as monoclonal antibody therapy, in which antibodies are designed to bind to specific CD antigens and trigger an immune response against cancer cells or infected cells. They can also be used as markers to monitor the effectiveness of treatments and to detect minimal residual disease (MRD) after treatment.
It's important to note that not all CD antigens are exclusive to leukocytes, some can be found on other cell types as well, and their expression can vary depending on the activation state or differentiation stage of the cells.
T-lymphocyte subsets refer to distinct populations of T-cells, which are a type of white blood cell that plays a central role in cell-mediated immunity. The two main types of T-lymphocytes are CD4+ and CD8+ cells, which are defined by the presence or absence of specific proteins called cluster differentiation (CD) molecules on their surface.
CD4+ T-cells, also known as helper T-cells, play a crucial role in activating other immune cells, such as B-lymphocytes and macrophages, to mount an immune response against pathogens. They also produce cytokines that help regulate the immune response.
CD8+ T-cells, also known as cytotoxic T-cells, directly kill infected cells or tumor cells by releasing toxic substances such as perforins and granzymes.
The balance between these two subsets of T-cells is critical for maintaining immune homeostasis and mounting effective immune responses against pathogens while avoiding excessive inflammation and autoimmunity. Therefore, the measurement of T-lymphocyte subsets is essential in diagnosing and monitoring various immunological disorders, including HIV infection, cancer, and autoimmune diseases.
Flow cytometry is a medical and research technique used to measure physical and chemical characteristics of cells or particles, one cell at a time, as they flow in a fluid stream through a beam of light. The properties measured include:
* Cell size (light scatter)
* Cell internal complexity (granularity, also light scatter)
* Presence or absence of specific proteins or other molecules on the cell surface or inside the cell (using fluorescent antibodies or other fluorescent probes)
The technique is widely used in cell counting, cell sorting, protein engineering, biomarker discovery and monitoring disease progression, particularly in hematology, immunology, and cancer research.
Respiratory tract infections (RTIs) are infections that affect the respiratory system, which includes the nose, throat (pharynx), voice box (larynx), windpipe (trachea), bronchi, and lungs. These infections can be caused by viruses, bacteria, or, less commonly, fungi.
RTIs are classified into two categories based on their location: upper respiratory tract infections (URTIs) and lower respiratory tract infections (LRTIs). URTIs include infections of the nose, sinuses, throat, and larynx, such as the common cold, flu, laryngitis, and sinusitis. LRTIs involve the lower airways, including the bronchi and lungs, and can be more severe. Examples of LRTIs are pneumonia, bronchitis, and bronchiolitis.
Symptoms of RTIs depend on the location and cause of the infection but may include cough, congestion, runny nose, sore throat, difficulty breathing, wheezing, fever, fatigue, and chest pain. Treatment for RTIs varies depending on the severity and underlying cause of the infection. For viral infections, treatment typically involves supportive care to manage symptoms, while antibiotics may be prescribed for bacterial infections.
Lymphoproliferative disorders (LPDs) are a group of diseases characterized by the excessive proliferation of lymphoid cells, which are crucial components of the immune system. These disorders can arise from both B-cells and T-cells, leading to various clinical manifestations ranging from benign to malignant conditions.
LPDs can be broadly classified into reactive and neoplastic categories:
1. Reactive Lymphoproliferative Disorders: These are typically triggered by infections, autoimmune diseases, or immunodeficiency states. They involve an exaggerated response of the immune system leading to the excessive proliferation of lymphoid cells. Examples include:
* Infectious mononucleosis (IM) caused by Epstein-Barr virus (EBV)
* Lymph node enlargement due to various infections or autoimmune disorders
* Post-transplant lymphoproliferative disorder (PTLD), which occurs in the context of immunosuppression following organ transplantation
2. Neoplastic Lymphoproliferative Disorders: These are malignant conditions characterized by uncontrolled growth and accumulation of abnormal lymphoid cells, leading to the formation of tumors. They can be further classified into Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL). Examples include:
* Hodgkin lymphoma (HL): Classical HL and nodular lymphocyte-predominant HL
* Non-Hodgkin lymphoma (NHL): Various subtypes, such as diffuse large B-cell lymphoma, follicular lymphoma, mantle cell lymphoma, and Burkitt lymphoma
It is important to note that the distinction between reactive and neoplastic LPDs can sometimes be challenging, requiring careful clinical, histopathological, immunophenotypic, and molecular evaluations. Proper diagnosis and classification of LPDs are crucial for determining appropriate treatment strategies and predicting patient outcomes.
Lymphocyte subsets refer to distinct populations of white blood cells called lymphocytes, which are crucial components of the adaptive immune system. There are two main types of lymphocytes: T cells and B cells, and each type has several subsets based on their surface receptors, functions, and activation status.
1. T cell subsets: These include CD4+ T helper cells (Th cells), CD8+ cytotoxic T cells (Tc cells), regulatory T cells (Tregs), and memory T cells. Th cells are further divided into Th1, Th2, Th17, and Tfh cells based on their cytokine production profiles and functions.
* CD4+ T helper cells (Th cells) play a central role in orchestrating the immune response by producing various cytokines that activate other immune cells.
* CD8+ cytotoxic T cells (Tc cells) directly kill virus-infected or malignant cells upon recognition of specific antigens presented on their surface.
* Regulatory T cells (Tregs) suppress the activation and proliferation of other immune cells to maintain self-tolerance and prevent autoimmunity.
* Memory T cells are long-lived cells that remain in the body after an initial infection or immunization, providing rapid protection upon subsequent encounters with the same pathogen.
2. B cell subsets: These include naïve B cells, memory B cells, and plasma cells. Upon activation by antigens, B cells differentiate into antibody-secreting plasma cells that produce specific antibodies to neutralize or eliminate pathogens.
* Naïve B cells are resting cells that have not yet encountered their specific antigen.
* Memory B cells are long-lived cells generated after initial antigen exposure, which can quickly differentiate into antibody-secreting plasma cells upon re-exposure to the same antigen.
* Plasma cells are terminally differentiated B cells that secrete large amounts of specific antibodies.
Analyzing lymphocyte subsets is essential for understanding immune system function and dysfunction, as well as monitoring the effectiveness of immunotherapies and vaccinations.
HIV (Human Immunodeficiency Virus) infection is a viral illness that progressively attacks and weakens the immune system, making individuals more susceptible to other infections and diseases. The virus primarily infects CD4+ T cells, a type of white blood cell essential for fighting off infections. Over time, as the number of these immune cells declines, the body becomes increasingly vulnerable to opportunistic infections and cancers.
HIV infection has three stages:
1. Acute HIV infection: This is the initial stage that occurs within 2-4 weeks after exposure to the virus. During this period, individuals may experience flu-like symptoms such as fever, fatigue, rash, swollen glands, and muscle aches. The virus replicates rapidly, and the viral load in the body is very high.
2. Chronic HIV infection (Clinical latency): This stage follows the acute infection and can last several years if left untreated. Although individuals may not show any symptoms during this phase, the virus continues to replicate at low levels, and the immune system gradually weakens. The viral load remains relatively stable, but the number of CD4+ T cells declines over time.
3. AIDS (Acquired Immunodeficiency Syndrome): This is the most advanced stage of HIV infection, characterized by a severely damaged immune system and numerous opportunistic infections or cancers. At this stage, the CD4+ T cell count drops below 200 cells/mm3 of blood.
It's important to note that with proper antiretroviral therapy (ART), individuals with HIV infection can effectively manage the virus, maintain a healthy immune system, and significantly reduce the risk of transmission to others. Early diagnosis and treatment are crucial for improving long-term health outcomes and reducing the spread of HIV.
Common variable immunodeficiency
Immunoglobulin therapy
Neurosarcoidosis
Selective immunoglobulin A deficiency
Thymoma with immunodeficiency
Granulomatous-lymphocytic interstitial lung disease
Suranjith Seneviratne
Transmembrane activator and CAML interactor
Roberto Paganelli
Danger model
CD278
Hypogammaglobulinemia
Wolf-Hirschhorn syndrome
Mycoplasma orale
Actinomyces bovis
Behçet's disease
IgG deficiency
NFKB2
HLA DR3-DQ2
DAVID syndrome
Hyper IgM syndrome
Plasma cell
Lymphoproliferative disorders
Lymphocyte
Infectious causes of cancer
Retinal vasculitis
SWAP70
Apilimod
LPS-responsive beige-like anchor protein deficiency
Hyaluronidase
Common variable immunodeficiency - Wikipedia
Common Variable Immunodeficiency: Practice Essentials, Background, Pathophysiology
Pediatric Common Variable Immunodeficiency Clinical Presentation: History, Physical, Causes
Pediatric Common Variable Immunodeficiency Workup: Laboratory Studies, Imaging Studies, Other Tests
Efficacy of intravenous immunoglobulin treatment in children with common variable immunodeficiency
West Nile Virus Meningitis in Patient with Common Variable Immunodeficiency - Volume 9, Number 10-October 2003 - Emerging...
Disseminated bronchiectasis in an adult with Common Variable Immunodeficiency | Colombia Medica
Abatacept for the Treatment of Common Variable Immunodeficiency With Interstitial Lung Disease | Pulmonary Fibrosis Foundation
Common Variable Immunodeficiency (CVID) - Vejthani Hospital | JCI Accredited International Hospital in Bangkok, Thailand.
The Investigation of Autoimmunity and B Cell Characteristics in Common Variable Immunodeficiency Patients - ...
Fusarium ramigenum, a novel human opportunist in a patient with common variable immunodeficiency and cellular immune defects:...
Pediatric Common Variable Immunodeficiency Medication
Spectrum of lymphoproliferation in common variable immunodeficiency disorders - review of the literature - Nuffield Department...
The variable in common variable immunodeficiency: A disease of complex phenotypes -ORCA
Update on Immunodeficiency-Associated Vaccine-Derived Polioviruses - Worldwide, July 2018-December 2019 | MMWR
Fact file for Common variable immunodeficiency of unknown origin
Common Variable Immunodeficiency (CVID) - Immune Disorders - MSD Manual Consumer Version
Helping patients | Institute of Immunity and Transplantation - UCL - University College London
Common variable immunodeficiency in vitiligo. | Read by QxMD
Pathogenesis of immune thrombocytopenia in common variable immunodeficiency
Could You Have Common Variable Immunodeficiency? • Dr. Drew Huffman
Natural cure for Common Variable Immunodeficiency and alternative treatments
The 2017 IUIS Phenotypic Classification for Primary Immunodeficiencies | Journal of Clinical Immunology
Infection outcomes in patients with common variable immunodeficiency disorders: relationship to immunoglobulin therapy over 22...
Common variable immune deficiency: MedlinePlus Genetics
University of California Health Common Variable Immunodeficiency Clinical Trials for 2023 - California
Conditions & Treatments Guide | St. Louis Children's Hospital
Primary immunodeficiency: Research & Insights | WEHI
Holiday Gathering - The Painted Turtle
ESID - European Society for Immunodeficiencies
CVID33
- Common variable immunodeficiency (CVID) is an immune disorder characterized by recurrent infections and low antibody levels, specifically in immunoglobulin (Ig) types IgG, IgM, and IgA. (wikipedia.org)
- Common infections include: Pneumonia Ear infections Sinusitis Chronic coughing (lasting from a few weeks to many months) Gastrointestinal infections Gastrovascular infections or inflammation are very common for those with CVID. (wikipedia.org)
- Lymphoma is a common cause of death in patients with CVID. (medscape.com)
- Common variable immunodeficiency (CVID), the most common clinical primary immunodeficiency in adults, is a disorder that involves the following: (1) low levels of most or all of the immunoglobulin (Ig) classes, (2) a lack of B lymphocytes or plasma cells that are capable of producing antibodies, and (3) frequent bacterial infections. (medscape.com)
- Clinical manifestations of common variable immunodeficiency (CVID) include recurrent infections, autoimmune disease, lymphoid hyperplasia, granulomatous diseases, and malignancy. (medscape.com)
- A diagnosis of common variable immunodeficiency (CVID) is based on defective functional antibody formation, usually accompanied by decreased (not absent) serum immunoglobulin (Ig)G and IgA levels, generally (not invariably) decreased serum IgM, and exclusion of other known causes of antibody deficiency. (medscape.com)
- Children with common variable immunodeficiency (CVID) have increased susceptibility to infections. (nih.gov)
- In September 2002, during a WNV epidemic in Michigan ( 2 ), a 38-year-old woman with common variable immunodeficiency (CVID) sought treatment at the University of Michigan Hospital with acute WNV-associated meningitis. (cdc.gov)
- Primary immunodeficiency (PID) hypogammaglobulinemia Common Variable Immunodeficiency (CVID) bronchiectasis Recurring pneumonia. (univalle.edu.co)
- Antibody deficiencies, especially Common Variable Immunodeficiency (CVID), which have their peak incidence in adulthood, require a high suspicion index. (univalle.edu.co)
- Common Variable Immunodeficiency (CVID) diagnosis was made. (univalle.edu.co)
- Clinical and Immunological Spectrum of Common Variable Immunodeficiency (CVID). (univalle.edu.co)
- Common Variable Immune Deficiency (CVID) is characterized by hypogammaglobulinemia and impaired antibody responses to vaccination. (ucl.ac.uk)
- Common variable immunodeficiency (CVID) is the most common and clinically most important severe primary antibody deficiency and is characterized by low levels of IgG, IgA, and/or IgM, with a failure to produce specific antibodies. (cardiff.ac.uk)
- Most of these present in childhood but the most common of all PIDs occurs in adults - currently known as 'Common Variable Immunodeficiency' (CVID). (ucl.ac.uk)
- Three cases of adult, non-segmental vitiligo patients in which primary, common variable immunodeficiency (CVID) was present are described. (qxmd.com)
- Common Variable Immunodeficiency (CVID) is the most common Primary Immunodeficiency seen in clinical practice. (univr.it)
- About 25-30% of CVID patients are affected by autoimmune manifestation, among which ITP is the most common. (univr.it)
- According to our results, we may suggest that in CVID patients, ITP is due to a decreased bone marrow platelets production, rather than an increased peripheral platelet destruction, which is more common in patients with primary ITP. (univr.it)
- Common Variable Immunodeficiency (or CVID) describes a condition that is characterized by a person having low immunoglobulins (immune - o - glob- u - lins). (drdrewhuffman.com)
- CVID is not common and occurs at a rate of 1:10,000 to 1:50,000 people. (drdrewhuffman.com)
- The cure of Common Variable Immunodeficiency (CVID) is quite similar to the treatment administered by low levels of serum immunoglobulins. (naturalcurefor.com)
- Common variable immune deficiency (CVID) is a disorder that impairs the immune system. (medlineplus.gov)
- Pneumonia is common in people with CVID. (medlineplus.gov)
- There is no standard of care therapy for patients with granulomatous-lymphocytic interstitial lung disease (GLILD) seen in common variable immunodeficiency (CVID). (ucbraid.org)
- Overview Common variable immunodeficiency (CVID) is an immunodeficiency problem that causes a child to have a low level of antibodies and a decreased responsiveness to some vaccines. (stlouischildrens.org)
- Common variable immunodeficiency (CVID) is the most common primary immunodeficiency, both in Australia and worldwide. (edu.au)
- Diagnosing CVID is difficult and requires exclusion of other primary immunodeficiencies. (edu.au)
- Common variable immunodeficiency (CVID) is a clinically, genetically and immunologically heterogeneous primary immunodeficiency disease (PID). (mayo.edu)
- These immune deficiency diseases are thought to be rare and include: Severe combined immunodeficiency (SCID), leukocyte adhesion deficiency (LAD), X-linked Agammaglobulinemia (XLA), common variable immune deficiency (CVID), DiGeorge syndrome (DGS), Hyper IgM syndrome (HIGM), Wiskott Aldrich syndrome (WAS) and chronic granulomatous disease (CGD). (mayo.edu)
- The study included participants with common variable immunodeficiency (CVID), which is rarely monogenic. (cdc.gov)
- Autoimmune lymphoproliferative syndrome (ALPS) in a patient with common variable immunodeficiency (CVID): Fas deficiency with increased apoptosis. (yumaregional.org)
- Recently completed molecular studies of poliovirus isolates suggest that viral replication of vaccine-related polioviruses may have persisted for as long as 7 years in a patient with vaccine-associated paralytic poliomyelitis (VAPP) in whom common variable immunodeficiency syndrome (CVID) previously had been diagnosed. (cdc.gov)
Diagnosis4
- Global study of primary immunodeficiency diseases (PI)--diagnosis, treatment, and economic impact: an updated report from the Jeffrey Modell Foundation. (univalle.edu.co)
- Our research seeks to discover how immunodeficiencies occur, with the goals of improving diagnosis of immunodeficiencies and how they are treated. (edu.au)
- The samples collected in this study will provide a valuable and unique sample database that will permit formulation of research protocols aimed at evaluating the biological underpinnings of immune competence and function in PID and the development of novel methods for the assessment of immune parameters in diagnosis of these immunodeficiency diseases. (mayo.edu)
- This note explains Common Variable Immunodeficiency signs and symptoms, diagnosis and management. (freebookcentre.net)
Patient with common variable immunod2
- Autoimmune lymphoproliferative syndrome in a patient with common variable immunodeficiency: dichotomy of apoptosis. (yumaregional.org)
- Ulcerative colitis in a patient with common variable immunodeficiency: does the treatment differ from the routine? (bvsalud.org)
SCID2
- Patients present with recurrent infections associated with T-lymphocytopenia, characteristically presenting later than patients with classical severe combined immunodeficiency (SCID). (frontiersin.org)
- Patients present with predominantly T-lymphocytopenia, and the immunological presentation characteristically is later than patients with classical severe combined immunodeficiency (SCID) ( 4 ). (frontiersin.org)
Disorders16
- Common variable immunodeficiency disorders (CVIDs) represent a group of primary immunodeficiency disorders, characterized by a deficit of antibody production although other aspects of the immune system are frequently altered too. (ox.ac.uk)
- Overview of Immunodeficiency Disorders Immunodeficiency disorders involve malfunction of the immune system, resulting in infections that develop and recur more frequently, are more severe, and last longer than usual. (msdmanuals.com)
- Infection outcomes in patients with common variable immunodeficiency disorders: relationship to immunoglobulin therapy over 22 years. (ox.ac.uk)
- BACKGROUND: Common variable immunodeficiency disorders (CVIDs) are the most common forms of symptomatic primary antibody failure in adults and children. (ox.ac.uk)
- Lymphoid proliferations of indeterminate malignant potential arising in adults with common variable immunodeficiency disorders: unusual case studies and immunohistological review in the light of possible causative events. (ox.ac.uk)
- Patients with common variable immunodeficiency disorders (CVIDs) who developed B cell lymphoproliferation of indeterminate malignant potential are described in order to raise a discussion of the relationship between infection and lymphoproliferation in infection prone patients. (ox.ac.uk)
- Key stages of bone marrow B-cell maturation are defective in patients with common variable immunodeficiency disorders. (ox.ac.uk)
- Primary immunodeficiencies (PI) are a group of more than 400 genetic disorders that alter the ability of the immune system to fight off infection and affect 1 out of 1,200 1 births in the United States. (cdc.gov)
- Topics covered includes: What is dermatology, Essential Clinical skills, Emergency Dermatology, Skin Infections and Infestations, Skin Cancer, Inflammatory Skin Conditions, Blistering Disorders and Common Important Problems. (freebookcentre.net)
- Immunodeficiency disorders affect the immune system's natural ability to defend the body against infection. (cancer.ca)
- People with immunodeficiency disorders often have frequent infections, which can be severe or unusual. (cancer.ca)
- Immunodeficiency disorders can also develop over the course of your life. (cancer.ca)
- These are called acquired immunodeficiency disorders. (cancer.ca)
- Inherited immunodeficiency disorders are very rare, but acquired ones are a little more common. (cancer.ca)
- The following immunodeficiency disorders increase the risk of developing NHL. (cancer.ca)
- Immunodeficiency-related lymphoproliferative disorders (LPDs) form a heterogeneous group that ranges from self-limiting hyperplasias to aggressive lymphomas. (medscape.com)
Cause common variable immunod2
- The genetic mutations that cause common variable immunodeficiency can be inherited, but more often, they occur spontaneously. (msdmanuals.com)
- Discovering the genetic and immune changes that cause common variable immunodeficiency, the most common inherited (primary) immunodeficiency in Australia and worldwide. (edu.au)
Severe5
- Severe and recurrent infections with herpes simplex are common, and herpes zoster eventually develops in as many as 20% of patients with common variable immunodeficiency. (medscape.com)
- Detailed immunological assessment revealed the presence of common variable immunodeficiency, complicated by a severe impairment of the capacity of T-cells to produce both gamma-interferon and interleukin-17. (biomedcentral.com)
- Patients with more profound immunodeficiency or patients with active infections have more severe reactions. (medscape.com)
- a Severe combined immunodeficiencies defined by T cell lymphopenia. (springer.com)
- Homozygous autosomal recessive mutations in RTEL1 lead to similar phenotypes that parallel with Hoyeraal-Hreidarsson (HH) syndrome, a severe variant of DKC characterized by cerebellar hypoplasia, bone marrow failure, intrauterine growth restriction and immunodeficiency. (medscape.com)
Infections9
- Variable" refers to the heterogeneous clinical manifestations of this disorder, which include recurrent bacterial infections, increased risk for autoimmune disease and lymphoma, as well as gastrointestinal disease. (wikipedia.org)
- As with any antibody deficiency, the most common types of infections and illnesses involve the ears, sinuses, nose, and lungs. (wikipedia.org)
- People with common variable immunodeficiency have trouble fighting off infections due to the lack of antibodies produced, which normally resist invading microbes. (wikipedia.org)
- Patients with agammaglobulinemia, either common variable or X-linked, are known to be susceptible to recurrent infections ( 3 ). (cdc.gov)
- People with common variable immunodeficiency may have a chronic cough, cough up blood, or have difficulty breathing (due to frequent sinus and lung infections) and may have diarrhea. (msdmanuals.com)
- Common Variable Immunodeficiency is type of disorder that is characterized by extremely low levels of serum immunoglobulin or what is known as antibodies as well as the increased susceptibility to infections. (naturalcurefor.com)
- A person with an immunodeficiency has trouble fighting infections. (edu.au)
- Primary immunodeficiencies (PID) are diseases that affect any part of multiple components of the immune system, resulting in abnormal and/or impaired immune responses and increased susceptibility to life threatening infections, autoimmune disease and neoplasias. (mayo.edu)
- Urinary tract infections (UTIs) are among the most common bacterial infections. (microbiologyresearch.org)
Deficiency5
- IgG subclass determination is of limited value in assessing patients with clinical immunodeficiency because functional antibody deficiency may be present despite normal IgG subclass levels. (medscape.com)
- Cunningham-Rundles C. How I treat common variable immune deficiency. (univalle.edu.co)
- Purine nucleoside phosphorylase (PNP) deficiency, a rare autosomal recessive metabolic disease causes combined immunodeficiency and developmental delay, hypotonia, and spasticity. (frontiersin.org)
- Purine nucleoside phosphorylase (PNP) deficiency (OMIM 613179) is a rare autosomal recessive metabolic disease leading to combined immunodeficiency and neurological abnormalities, which may include developmental delay, hypotonia, and spasticity ( 1 , 2 ). (frontiersin.org)
- Purine nucleoside phosphorylase is a ubiquitously expressed enzyme of the purine salvage pathway as is adenosine deaminase, deficiency of which also causes immunodeficiency. (frontiersin.org)
Primary immunodeficiency diseases1
- Riminton DS, Limaye S. Primary immunodeficiency diseases in adulthood. (univalle.edu.co)
Infection9
- Recurrent infection was the only presenting symptom in 26% of patients reported by the European common variable immunodeficiency registry. (medscape.com)
- Fusarium species are common saprophytic fungi that globally represent the third cause of invasive mould infection in humans, after Aspergillus and after Mucorales. (biomedcentral.com)
- This opportunistic infection is common in Brazil but rare in other parts of the world. (biomedcentral.com)
- The iVDPV case definition is a laboratory-confirmed VDPV infection in a person of any age who has a primary humoral (B-cell) or combined humoral and cellular (B- and T-cell) immunodeficiency disorder ( 6 ). (cdc.gov)
- The common cold (upper respiratory infection) is one of the most common illnesses in children as most will have at least 6 to 8 colds a year. (stlouischildrens.org)
- Secondary immunodeficiency describes immunodeficiencies arising as the result of another event such as HIV infection or taking immune suppressing medication. (edu.au)
- Chronic mucocutaneous candidiasis, a hereditary immunodeficiency disorder, is persistent or recurring infection with Candida (a fungus) due to malfunction of T cells (a type of white blood cell). (merckmanuals.com)
- The most common type of candidiasis is a superficial infection of. (merckmanuals.com)
- 8. Positive serological tests for hepatitis B, hepatitis C (also confirmed with HCV RNA), or human immunodeficiency virus (HIV) infection, or known infection status at Visit 2. (who.int)
Immunoglobulins1
- These mechanisms implicate both the constant (Fc) and the variable region (Fab') of the immunoglobulins. (academie-medecine.fr)
Interstitial Lung Di2
- Granulomatous and lymphocytic interstitial lung disease: a spectrum of pulmonary histopathologic lesions in common variable immunodeficiency-histologic and immunohistochemical analyses of 16 cases. (stanford.edu)
- Approximately 10-30% of patients with common variable immunodeficiency develop clinical evidence of a diffuse parenchymal lung disease with a constellation of histopathologic findings termed granulomatous and lymphocytic interstitial lung disease. (stanford.edu)
PIDs1
- Immunodeficiency-associated vaccine-derived polioviruses (iVDPVs) emerge among persons with primary immunodeficiencies (PIDs) and rarely can persist. (cdc.gov)
Genetic1
- The causes of the common variable immunodeficiency are linked to genetic factors as well as the prolonged use of antiepileptic drugs. (naturalcurefor.com)
Immunoglobulin therapy1
- Cunningham-Rundles C. Key aspects for successful immunoglobulin therapy of primary immunodeficiencies. (univalle.edu.co)
Adults3
- Kumar Y, Bhatia A. Comment on Common variable immunodeficiency in adults: current diagnostic protocol and laboratory measures. (univalle.edu.co)
- It is the most frequently occurring symptomatic primary immunodeficiency encountered in adults. (drdrewhuffman.com)
- This PDF covers the following topics related to Dermatology : The structure and function of the skin, Taking a history, Examining the skin and describing lesions, Common skin conditions in adults, Common skin conditions in both adults and children, Practical advice on topical treatments. (freebookcentre.net)
Agammaglobulinemia2
- In contrast to X-linked agammaglobulinemia (XLA), common variable immunodeficiency is associated with a high frequency of autoimmune manifestation. (medscape.com)
- Serum Ig levels are consistently depressed but are generally higher in patients with common variable immunodeficiency than in patients with X-linked agammaglobulinemia. (medscape.com)
Paralytic1
- The case described in this report is exceptional because it is the only known VAPP case in an immunodeficient person in which immunodeficiency had been diagnosed before onset of paralytic manifestations (5). (cdc.gov)
Deficiencies1
- The Immune Deficiencies Foundation Australia provides information and support for people with immunodeficiencies, their carers and relatives. (edu.au)
Lymphoma1
- The two most common cancers in patients with common variable immunodeficiency include lymphoma and certain stomach cancers. (wikipedia.org)
Heterogeneous group1
- Common variable immunodeficiency is a heterogeneous group of diseases of unknown etiology. (lu.se)
Diseases2
- Other solid organ-specific autoimmune diseases (eg, pernicious anemia, thyroid diseases, vitiligo) have prevalence rates of more than 5% in patients with common variable immunodeficiency, which is higher than in the general population. (medscape.com)
- Conceived initially for antibody replacement therapy in patients with primary and secondary immunodeficiencies, IVIG is now used in several autoimmune and inflammatory diseases. (academie-medecine.fr)
Manifestations1
- Fernandez Romero DS, Juri MC, Paolini MV, Malbran A. Common variable immunodeficiency: Epidemiology and clinical manifestations in 69 patients. (univalle.edu.co)
Immunology1
- Our immunology researchers aim to understand how immunodeficiencies occur, and how they can be better treated. (edu.au)
Pneumonia1
- Pneumonia is one of the most common causes of death worldwide. (msdmanuals.com)
Syndrome1
- People with HIV (human immunodeficiency virus) or AIDS (acquired immunodeficiency syndrome) have a greater risk of developing some types of NHL. (cancer.ca)
Spectrum1
- These cases offered the opportunity to evaluate and compare a wide spectrum of immunodeficiency-related LPDs, assess progress in the field, build on the current understanding and approach to classification, and explore areas for future study. (medscape.com)
Autoimmune disease1
- Immune Thrombocitopenic Purpura (ITP) is an autoimmune disease characterized by antibody-mediated platelet destruction and variable reduced platelet production. (univr.it)
Humoral1
- Immunodeficiencies affecting cellular and humoral immunity. (springer.com)
Classification1
- Classification of common variable immunodeficiencies using flow cytometry and a memory B-cell functionality assay. (univalle.edu.co)
Pulmonary1
- Pulmonary Hypertension in Patients with Common Variable Immunodeficiency. (nih.gov)
Genetically1
- and 3) ambiguous VDPVs (aVDPVs), when isolated from persons with no known immunodeficiency and when there is no evidence of community transmission or when isolates from sewage are not genetically linked to other known VDPVs and whose source is unknown ( 3 ). (cdc.gov)
Antibodies2
- Common variable immunodeficiency is an immunodeficiency disorder characterized by very low antibody (immunoglobulin) levels despite a normal number of B cells (the type of white blood cell that makes antibodies). (msdmanuals.com)
- Common variable immunodeficiency is a primary immunodeficiency of unknown etiology characterized by low serum immunoglobulin G, a decreased ability to make specific antibodies, and variable T-cell defects. (stanford.edu)
Persistent1
- Persistent diarrhea and malabsorption caused by Giardia lamblia also have been reported in patients with common variable immunodeficiency. (medscape.com)
Incidence1
- Thymomas are not common neoplasias, with an incidence of 0.15 cases per 100,000 people per year. (ecancer.org)
Registry1
- A 'Registry' is a list of basic information about people who have a certain disease or condition in common. (mayo.edu)
Patients with common4
- The risk for these given cancers is almost fifty times greater in patients with common variable immunodeficiency than in those without. (wikipedia.org)
- A few patients with common variable immunodeficiency present with unusual organisms, such as Pneumocystis jiroveci , mycobacteria, or various fungi. (medscape.com)
- The most common autoimmune conditions in patients with common variable immunodeficiency are cytopenia, idiopathic thrombocytopenic purpura (ITP) in particular, and hemolytic anemia or, more rarely, autoimmune neutropenia. (medscape.com)
- Atypical lymphoid hyperplasia due to clonal expansion of B or T lymphocytes has been reported in as many as one third of patients with common variable immunodeficiency. (medscape.com)