Immunoproliferative Disorders
Immunoproliferative Small Intestinal Disease
Lymphomatoid Granulomatosis
Immunoglobulin alpha-Chains
Angiocentric immunoproliferative lesion and angiocentric lymphoma of lymph node in children. A report of two cases. (1/11)
AIM: To report two examples of an angiocentric immunoproliferative lesion (AIL) and angiocentric angiodestructive lymphoma (AL) presenting in lymph nodes in children. Most commonly involving extranodal sites, AIL/AL rarely presents in the spleen and lymph nodes. METHODS/RESULTS: Case 1 presented as a cervical lymphadenopathy in a 3 year old girl being treated for pre-B cell acute lymphoblastic leukaemia. Histological and immunohistochemistry studies revealed an Epstein-Barr virus positive (EBV+), large B cell (CD20 and CD30+) AIL with large areas of necrosis, the whole resembling lymphomatoid granulomatosis. Case 2 presented as a large supraclavicular lymphadenopathy in a 13 year old boy. Histology and immunohistochemistry revealed an EBV-, large T cell (CD45RO, CD56, and CD30+) AL, presenting the features of so called angiocentric T cell/natural killer cell lymphoma, nasal type. CONCLUSIONS: The term AIL/AL refers to a heterogeneous group of conditions not unique to a particular type of lymphoid cell. These lesions are easily recognised by the histopathologist because of their extremely unusual angiocentric pattern. Although rare, AIL/AL may present as nodal lesions in children ab initio. (+info)Targeted inhibition of beta-adrenergic receptor kinase-1-associated phosphoinositide-3 kinase activity preserves beta-adrenergic receptor signaling and prolongs survival in heart failure induced by calsequestrin overexpression. (2/11)
OBJECTIVES: Desensitization and down-regulation of beta-adrenergic receptors (betaARs) are prominent features of heart failure largely mediated by increased levels of betaAR kinase-1 (betaARK1). BACKGROUND: beta-adrenergic receptor kinase 1 interacts with phosphoinositide-3 kinase (PI3K), and upon agonist stimulation, the betaARK1/PI3K complex is recruited to agonist-stimulated betaARs. Here we tested the hypothesis that in vivo selective inhibition of betaARK1-associated PI3K activity would preserve betaAR signaling and, therefore, improve cardiac function and survival in experimental heart failure. METHODS: We used a murine model of heart failure induced by calsequestrin (CSQ) cardiac-specific overexpression; CSQ mice were crossed with mice overexpressing in the heart a catalytically inactive PI3Kgamma (PI3Kgamma(inact)) to competitively displace endogenous PI3K from betaARK1. RESULTS: Catalytically inactive PI3KgammaPI3K overexpression in CSQ mice inhibited betaARK1-associated PI3K activity, normalized betaAR levels, and preserved betaAR responsiveness to isoproterenol (ISO). Restoration of betaAR signaling via PI3Kgamma(inact) overexpression resulted in marked improvement of cardiac function and a significant prolongation of survival. Importantly, the effects of PI3Kgamma(inact) overexpression were restricted to betaAR signaling, because cellular PI3K signaling was unaltered, as shown by the similar activation of multiple downstream signaling pathways in both CSQ and CSQ/PI3Kgamma(inact) mice. CONCLUSIONS: These data in the CSQ model of cardiac dysfunction indicate that membrane-targeted PI3K activity plays a detrimental role in heart failure, and its inhibition represents a novel therapeutic approach to ameliorate cardiac dysfunction and improve survival. (+info)Screening panels for detection of monoclonal gammopathies. (3/11)
(+info)RANKL induces heterogeneous DC-STAMP(lo) and DC-STAMP(hi) osteoclast precursors of which the DC-STAMP(lo) precursors are the master fusogens. (4/11)
(+info)Somatic KRAS mutations associated with a human nonmalignant syndrome of autoimmunity and abnormal leukocyte homeostasis. (5/11)
(+info)Morphologic features of extrahepatic manifestations of hepatitis C virus infection. (6/11)
(+info)CD200 expression in plasma cells of nonmyeloma immunoproliferative disorders: clinicopathologic features and comparison with plasma cell myeloma. (7/11)
(+info)The memory-enhancing effects of hippocampal estrogen receptor activation involve metabotropic glutamate receptor signaling. (8/11)
(+info)Immunoproliferative disorders are a group of conditions characterized by the abnormal proliferation (overgrowth) of immune cells, leading to the production of large numbers of abnormal antibodies or immunoglobulins. These disorders can result from genetic mutations, infections, autoimmune processes, or malignancies.
Examples of immunoproliferative disorders include:
1. Monoclonal gammopathy of undetermined significance (MGUS): A condition where a single clone of plasma cells produces an abnormal amount of one type of immunoglobulin, which can be detected in the blood. MGUS is not cancerous but may progress to multiple myeloma or other related disorders.
2. Multiple myeloma: A malignant proliferation of a single clone of plasma cells that produce large amounts of abnormal immunoglobulins, leading to bone destruction, anemia, infections, and kidney damage.
3. Waldenström macroglobulinemia: A rare type of lymphoplasmacytic lymphoma where a single clone of B-lymphocytes produces large amounts of abnormal immunoglobulins called IgM, leading to symptoms such as anemia, fatigue, bleeding, and neurological problems.
4. Cryoglobulinemia: A condition characterized by the presence of cryoglobulins (abnormal immunoglobulins) in the blood that precipitate at low temperatures, causing inflammation and damage to small blood vessels.
5. Amyloidosis: A rare disorder where abnormal proteins called amyloid deposits accumulate in various organs and tissues, leading to their dysfunction. In some cases, these proteins are derived from immunoglobulin light chains produced by clonal plasma cells.
Immunoproliferative disorders can have significant clinical consequences, including organ damage, impaired immune function, and increased risk of infections. Proper diagnosis and management require a multidisciplinary approach involving hematologists, oncologists, pathologists, and other specialists.
Immunoproliferative Small Intestinal Disease (IPSID) is a rare condition primarily affecting the small intestine. It is characterized by an excessive proliferation of immune cells, particularly plasma cells, in the lining of the small intestine. This leads to thickening of the intestinal wall, impaired absorption of nutrients, and various gastrointestinal symptoms. IPSID is often associated with a specific type of abnormal protein, called an alpha-defensin, in the stool. It's also known as alpha-defensin enteropathy or Mediterranean lymphoma. The exact cause of IPSID is not fully understood, but it may be linked to chronic antigenic stimulation, such as that caused by certain bacterial infections.
Lymphomatoid Granulomatosis is a rare, progressive and potentially fatal inflammatory disorder characterized by the proliferation of atypical B-lymphocytes infiltrating the lungs, skin, and less frequently other organs. It is considered an angiocentric and angiodestructive lymphoproliferative disease with varying degrees of malignancy. The condition is often associated with Epstein-Barr virus (EBV) infection and is more prevalent in middle-aged men.
The disorder presents with a wide range of symptoms, depending on the affected organs. Lung involvement can cause cough, shortness of breath, chest pain, or hemoptysis, while skin manifestations may include papules, nodules, or ulcers. Other possible sites of involvement are the central nervous system, kidneys, and liver.
Lymphomatoid Granulomatosis is classified into three grades based on the number of atypical lymphocytes and degree of necrosis: grade I (few atypical cells, minimal necrosis), grade II (more atypical cells, mild to moderate necrosis), and grade III (large numbers of atypical cells, extensive necrosis). Grades II and III are considered high-grade lymphomas.
The diagnosis is established through a combination of clinical presentation, radiological findings, and histopathological examination of biopsy specimens. Treatment options include corticosteroids, chemotherapy, immunomodulatory agents, and radiation therapy. The prognosis varies depending on the grade, extent of disease, and response to treatment.
Immunoglobulin alpha-chains (IgA) are a type of immunoglobulin or antibody that plays a crucial role in the immune system. They are composed of two heavy chains, known as alpha-chains, and two light chains. IgA is primarily found in secretions such as tears, saliva, breast milk, and respiratory and intestinal mucus, where they provide protection against pathogens that enter the body through these surfaces.
IgA can exist in two forms: a monomeric form, which consists of a single IgA molecule, and a polymeric form, which consists of several IgA molecules joined together by a J chain. The polymeric form is more common in secretions, where it provides an effective barrier against pathogens.
IgA functions by binding to antigens on the surface of pathogens, preventing them from attaching to and infecting host cells. It can also neutralize toxins produced by some bacteria and viruses. Additionally, IgA can activate the complement system, a group of proteins that work together to destroy pathogens, and initiate an immune response by recruiting other immune cells to the site of infection.
Deficiencies in IgA are relatively common and usually do not cause any significant health problems. However, in some cases, people with IgA deficiency may develop recurrent infections or allergies.
Malabsorption syndromes refer to a group of disorders in which the small intestine is unable to properly absorb nutrients from food, leading to various gastrointestinal and systemic symptoms. This can result from a variety of underlying conditions, including:
1. Mucosal damage: Conditions such as celiac disease, inflammatory bowel disease (IBD), or bacterial overgrowth that cause damage to the lining of the small intestine, impairing nutrient absorption.
2. Pancreatic insufficiency: A lack of digestive enzymes produced by the pancreas can lead to poor breakdown and absorption of fats, proteins, and carbohydrates. Examples include chronic pancreatitis or cystic fibrosis.
3. Bile acid deficiency: Insufficient bile acids, which are necessary for fat emulsification and absorption, can result in steatorrhea (fatty stools) and malabsorption. This may occur due to liver dysfunction, gallbladder removal, or ileal resection.
4. Motility disorders: Abnormalities in small intestine motility can affect nutrient absorption, as seen in conditions like gastroparesis, intestinal pseudo-obstruction, or scleroderma.
5. Structural abnormalities: Congenital or acquired structural defects of the small intestine, such as short bowel syndrome, may lead to malabsorption.
6. Infections: Certain bacterial, viral, or parasitic infections can cause transient malabsorption by damaging the intestinal mucosa or altering gut flora.
Symptoms of malabsorption syndromes may include diarrhea, steatorrhea, bloating, abdominal cramps, weight loss, and nutrient deficiencies. Diagnosis typically involves a combination of clinical evaluation, laboratory tests, radiologic imaging, and sometimes endoscopic procedures to identify the underlying cause. Treatment is focused on addressing the specific etiology and providing supportive care to manage symptoms and prevent complications.
Immunoproliferative disorder
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Lymphoproliferative disorders
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MeSH Browser
Lymphoproliferative5
- citation needed] These disorders are subdivided into three main classes, which are lymphoproliferative disorders, hypergammaglobulinemia, and paraproteinemia. (wikipedia.org)
- Lymphoproliferative disorders (LPDs) refer to several conditions in which lymphocytes are produced in excessive quantities. (wikipedia.org)
- An angiocentric and angiodestructive lymphoproliferative disorder primarily involving the lungs. (rush.edu)
- Heavy chain diseases are considered in patients with clinical manifestations suggesting lymphoproliferative disorders. (msdmanuals.com)
- which may facilitate the development of various lymphoproliferative disorders. (lookformedical.com)
Small intestinal3
- IgA heavy chain disease is the most common heavy chain disease and is sometimes called Mediterranean lymphoma (immunoproliferative small intestinal disease). (msdmanuals.com)
- Immunoproliferative Small Intestinal Disease. (booksdo.com)
- Immunoproliferative small intestinal disease - a genetic disease commonly seen in Basenji's, this inflammatory bowel disease creates severe diarrhea and vomiting in a dog that is accompanied by protein loss and malabsorption. (trained2listenk-9.com)
Characterized by abnormal proliferation3
- Disorders characterized by abnormal proliferation of primary cells of the immune system or by excessive production of immunoglobulins. (bvsalud.org)
- Disorders characterized by abnormal proliferation of immunoglobulin-producing cells and abnormal proliferation of immunoglobulin monoclonal (M protein)represent part of the spectrum of disease due to the neoplastic behavior of the B lymphocyte series. (emedicodiary.com)
- Paraproteinaemia is a disorder characterized by abnormal proliferation of immunoglobulin-producing cells due to the neoplastic behavior of B-Lymphocytic series with an increase in serum level of homogenous immunoglobulin (monoclonal IG) or its fragments. (emedicodiary.com)
Diseases3
- HHV-4, also known as Epstein-Barr virus (EBV), causes the primary infection infectious mononucleosis , and it is implicated in various diseases, such as African Burkitt lymphoma , other immunoproliferative disorders, and nasopharyngeal carcinoma. (medscape.com)
- Heavy chain diseases are neoplastic plasma cell disorders characterized by overproduction of monoclonal immunoglobulin heavy chains. (msdmanuals.com)
- Heavy chain diseases are plasma cell disorders that are typically malignant. (msdmanuals.com)
Monoclonal1
- In most plasma cell disorders, M-proteins (monoclonal immunoglobulin protein) are structurally similar to normal antibody molecules. (msdmanuals.com)
Disease1
- EN":"Only municipalities with at least 25000 residents have been included in the comparison"},"consulta_monografie":{"IT":"Consulta le monografie","EN":"View ULSS reports"},"dato_non_disponibile":{"IT":"Dato non disponibile","EN":"Not available data"},"disease_comparison":{"IT":"Confronto per sede tumorale. (registrotumoriveneto.it)
Autoimmune disorders1
- Funk RS, Shakhnovich V, Cho YK, Polireddy K, Jausurawong T, Gress K, Becker ML. Factors associated with reduced infliximab exposure in the treatment of pediatric autoimmune disorders: a cross-sectional prospective convenience sampling study. (childrensmercy.org)
Syndromes1
- Capsule?CAPD: Continuous ambulatory peritoneal dialysis?Caps: Capsules?CAPS: Cryopyrin-Associated Periodic Syndromes disorders?CAPTIA Syph G: ?CAPTIA Syph M: ?CAST: Cardiac arrhythmia suppression trials?CAT: Computerized axial tomography?Cataplasm. (kuwaitpharmacy.com)
Tissues1
- Disorders that are characterized by the production of antibodies that react with host tissues or immune effector cells that are autoreactive to endogenous peptides. (childrensmercy.org)
Proliferation3
- In immunology, immunoproliferative disorders are disorders of the immune system that are characterized by the abnormal proliferation of the primary cells of the immune system, which includes B cells, T cells and natural killer (NK) cells, or by the excessive production of immunoglobulins (also known as antibodies). (wikipedia.org)
- It is defined as a group of disorders arising from the abnormal proliferation of a single clone of immunoglobulin-secreting cells giving rise to paraproteinaemia. (emedicodiary.com)
- Overview of Plasma Cell Disorders Plasma cell disorders are a diverse group of disorders of unknown etiology characterized by Disproportionate proliferation of a single clone of B cells Presence of a structurally and electrophoretically. (msdmanuals.com)
Multiple1
- EN":"Exclusions: DCO, lost to follow-up, incompatible morfology or topografy according to 2016 AIRTUM monography 'La sopravvivenza dei pazienti oncologici in Italia', multiple tumours cases on the same site. (registrotumoriveneto.it)
Tree1
- Immunoproliferative disorders - Topic Tree. (wikipedia.org)
Types1
- There are 3 major types of Proliferative disorders. (emedicodiary.com)
Categories1
- These categories include acute traumatic injuries , aerodigestive disorders , cancers , mental health conditions , and musculoskeletal disorders . (wtcvictimfund.com)
Treatment1
- Symptoms, diagnosis, and treatment vary according to the specific disorder. (msdmanuals.com)
Small intestinal disease2
- This feature is seen in all of the lymphomas discussed above -- H pylori -associated marginal zone B-cell lymphoma, immunoproliferative small intestinal disease (Mediterranean lymphoma), Epstein Barr-associated posttransplant immunoproliferative disease, and enteropathy-associated T-cell lymphoma -- and was suspected in our patient's HCV-associated marginal zone B-cell lymphoma. (medscape.com)
- IgA heavy chain disease is the most common heavy chain disease and is sometimes called Mediterranean lymphoma (immunoproliferative small intestinal disease). (msdmanuals.com)
Lymphoma1
- A lymphoproliferative disorder characterized by pleomorphic B-LYMPHOCYTES including PLASMA CELLS, with increased levels of monoclonal serum IMMUNOGLOBULIN M. There is lymphoplasmacytic cells infiltration into bone marrow and often other tissues, also known as lymphoplasmacytic lymphoma. (uams.edu)
Gastrointestinal Diseases1
- The applications covered in the study include cancer, gastrointestinal diseases, immunoproliferative disorders, and others. (alliedmarketresearch.com)
Malignant1
- Heavy chain diseases are plasma cell disorders that are typically malignant. (msdmanuals.com)
Immunodeficiency3
- Screening immunoglobulin levels in CAP patients may also uncover underlying humoral immunodeficiency or immuno-proliferative disorders. (blogspot.com)
- Immune disorders such as hypersensitivity, immunodeficiency, autoimmunity, and protein abnormalities are addressed, as well as transplant and tumor immunology, immunologic testing methods and flow cytometry. (unmc.edu)
- The Immunology Laboratory is responsible for laboratory testing and clinical consultation in several broad areas including the evaluation of autoimmune disease, immunodeficiency, immunoproliferative disorders, and allergy, as well as having responsibility for some aspects of infectious disease serology. (asome.health)
Antibodies1
- In immunology, immunoproliferative disorders are disorders of the immune system that are characterized by the abnormal proliferation of the primary cells of the immune system, which includes B cells, T cells and natural killer (NK) cells, or by the excessive production of immunoglobulins (also known as antibodies). (wikipedia.org)
Lymphomatoid2
- A case of lymphomatoid granulomatosis /angiocentric immunoproliferative lesion with long clinical course and diffuse brain involvement. (lookfordiagnosis.com)
- lymphomatoid granulomatosis (LYG)/angiocentric immunoproliferative lesions (AIL) consist of angiocentric and angiodestructive lymphoreticular proliferation predominantly involving the lungs and other extranodal sites, such as the central nervous system (CNS). (lookfordiagnosis.com)
Immune1
- Disorders characterized by abnormal proliferation of primary cells of the immune system or by excessive production of immunoglobulins. (bvsalud.org)
Humoral1
- Disorders caused by abnormal or absent immunologic mechanisms, whether humoral, cell-mediated, or both. (umassmed.edu)
Lung3
- Angiocentric immunoproliferative lesions of the lung associated with diffuse renal involvement. (lookfordiagnosis.com)
- lung biopsy of segments 4 and 8 by video-assisted thorachoscopy revealed angiocentric and angiodestructive massive lymphoplasmocytic infiltrations with multinucleated giant cells , which were compatible with grade II angiocentric immunoproliferative lesions. (lookfordiagnosis.com)
- Angiocentric immunoproliferative lesion of the lung . (lookfordiagnosis.com)
Organs2
Diagnosis3
- We recommend the imaging of kidneys for diagnosis and following renal biopsy to evaluate the renal involvement of angiocentric immunoproliferative lesions. (lookfordiagnosis.com)
- Correlation of laboratory data with the diagnosis of erythrocyte, leukocyte and bleeding/clotting disorders will be introduced. (unmc.edu)
- Symptoms, diagnosis, and treatment vary according to the specific disorder. (msdmanuals.com)
Multiple1
- EN":"Exclusions: DCO, lost to follow-up, incompatible morfology or topografy according to 2016 AIRTUM monography 'La sopravvivenza dei pazienti oncologici in Italia', multiple tumours cases on the same site. (ict4life.com)
Plasma1
- In most plasma cell disorders, M-proteins (monoclonal immunoglobulin protein) are structurally similar to normal antibody molecules. (msdmanuals.com)
