A form of cutaneous T-cell lymphoma manifested by generalized exfoliative ERYTHRODERMA; PRURITUS; peripheral lymphadenopathy, and abnormal hyperchromatic mononuclear (cerebriform) cells in the skin, LYMPH NODES, and peripheral blood (Sezary cells).
A chronic, malignant T-cell lymphoma of the skin. In the late stages, the LYMPH NODES and viscera are affected.
A group of lymphomas exhibiting clonal expansion of malignant T-lymphocytes arrested at varying stages of differentiation as well as malignant infiltration of the skin. MYCOSIS FUNGOIDES; SEZARY SYNDROME; LYMPHOMATOID PAPULOSIS; and PRIMARY CUTANEOUS ANAPLASTIC LARGE CELL LYMPHOMA are the best characterized of these disorders.
The widespread involvement of the skin by a scaly, erythematous dermatitis occurring either as a secondary or reactive process to an underlying cutaneous disorder (e.g., atopic dermatitis, psoriasis, etc.), or as a primary or idiopathic disease. It is often associated with the loss of hair and nails, hyperkeratosis of the palms and soles, and pruritus. (From Dorland, 27th ed)
Tumors or cancer of the SKIN.
A characteristic symptom complex.
A KIR receptor that has specificity for HLA-A3 ANTIGEN. It is an inhibitory receptor that contains D0, D1, and D2 extracellular immunoglobulin-like domains and a long cytoplasmic tail.
Differentiation antigens expressed on pluripotential hematopoietic cells, most human thymocytes, and a major subset of peripheral blood T-lymphocytes. They have been implicated in integrin-mediated cellular adhesion and as signalling receptors on T-cells.
A KIR receptor that has specificity for HLA-C ANTIGENS. It is an inhibitory receptor that contains D1 and D2 extracellular immunoglobulin-like domains and a long cytoplasmic tail. It is similar in structure and function to the KIR2DL1 RECEPTORS and the KIR2DL3 RECEPTORS.
A process in which peripheral blood is exposed in an extracorporeal flow system to photoactivated 8-methoxypsoralen (METHOXSALEN) and ultraviolet light - a procedure known as PUVA THERAPY. Photopheresis is at present a standard therapy for advanced cutaneous T-cell lymphoma; it shows promise in the treatment of autoimmune diseases.
A serine protease that catalyses the release of an N-terminal dipeptide. Several biologically-active peptides have been identified as dipeptidyl peptidase 4 substrates including INCRETINS; NEUROPEPTIDES; and CHEMOKINES. The protein is also found bound to ADENOSINE DEAMINASE on the T-CELL surface and is believed to play a role in T-cell activation.
A lysosomal-associated membrane glycoprotein that is expressed at high levels in mature DENDRITIC CELLS.
Diseases of LYMPH; LYMPH NODES; or LYMPHATIC VESSELS.
Lymphocytes responsible for cell-mediated immunity. Two types have been identified - cytotoxic (T-LYMPHOCYTES, CYTOTOXIC) and helper T-lymphocytes (T-LYMPHOCYTES, HELPER-INDUCER). They are formed when lymphocytes circulate through the THYMUS GLAND and differentiate to thymocytes. When exposed to an antigen, they divide rapidly and produce large numbers of new T cells sensitized to that antigen.
Photochemotherapy using PSORALENS as the photosensitizing agent and ultraviolet light type A (UVA).
A potent inhibitor of ADENOSINE DEAMINASE. The drug induces APOPTOSIS of LYMPHOCYTES, and is used in the treatment of many lymphoproliferative malignancies, particularly HAIRY CELL LEUKEMIA. It is also synergistic with some other antineoplastic agents and has immunosuppressive activity.
Process of classifying cells of the immune system based on structural and functional differences. The process is commonly used to analyze and sort T-lymphocytes into subsets based on CD antigens by the technique of flow cytometry.
An intense itching sensation that produces the urge to rub or scratch the skin to obtain relief.
The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.
Ordered rearrangement of T-cell variable gene regions coding for the beta-chain of antigen receptors.
A group of heterogeneous lymphoid tumors representing malignant transformations of T-lymphocytes.
Technique using an instrument system for making, processing, and displaying one or more measurements on individual cells obtained from a cell suspension. Cells are usually stained with one or more fluorescent dyes specific to cell components of interest, e.g., DNA, and fluorescence of each cell is measured as it rapidly transverses the excitation beam (laser or mercury arc lamp). Fluorescence provides a quantitative measure of various biochemical and biophysical properties of the cell, as well as a basis for cell sorting. Other measurable optical parameters include light absorption and light scattering, the latter being applicable to the measurement of cell size, shape, density, granularity, and stain uptake.
Exfoliate neoplastic cells circulating in the blood and associated with metastasizing tumors.

Prognostic factors in primary cutaneous lymphomas other than mycosis fungoides and the Sezary syndrome. The French Study Group on Cutaneous Lymphomas. (1/261)

Prognostic studies of primary cutaneous lymphomas (PCL) other than mycosis fungoides (MF) and the Sezary syndrome (SS; non-MF/SS PCL) have been mainly performed on subgroups or on small numbers of patients by using univariate analyses. Our aim was to identify independent prognostic factors in a large series of patients with non-MF/SS PCL. We evaluated 158 patients who were registered in the French Study Group on Cutaneous Lymphomas database from January 1, 1986 to March 1, 1997. Variables analyzed for prognostic value were: age; sex; type of clinical lesions; maximum diameter, location, and number of skin lesions; cutaneous distribution (ie, local, regional, or generalized); prognostic group according to the European Organization for Research and Treatment of Cancer (EORTC) classification for PCL; B- or T-cell phenotype; serum lactate dehydrogenase (LDH) level; and B symptoms. Univariate and multivariate analyses were performed using a model of relative survival. Forty-nine patients (31%) died. The median relative survival time was 81 months. In univariate analysis, EORTC prognostic group, serum LDH level, B symptoms, and variables related to tumor extension (ie, distribution, maximum diameter, and number of skin lesions) were significantly associated with survival. When these variables were considered together in a multivariate analysis, EORTC prognostic group and distribution of skin lesions remained statistically significant, independent prognostic factors. This study confirms the good predictive value of the EORTC classification for PCL and shows that the distribution of skin lesions at initial evaluation is an important prognostic indicator.  (+info)

Ophthalmic abnormalities in patients with cutaneous T-cell lymphoma. (2/261)

PURPOSE: To determine the frequency of ophthalmic abnormalities in patients with cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome) and T-cell lymphoma involving the skin and to describe the clinical course of the disease with selected examples. METHODS: A computerized diagnostic retrieval system was used to identify all patients with T-cell lymphoma involving the skin who were examined at the Mayo Clinic (Rochester, Minnesota) between January 1, 1976 and December 31, 1990. The medical records of affected patients were reviewed. RESULTS: During the 15-year interval from 1976 through 1990, cutaneous T-cell lymphoma was diagnosed in 2,155 patients. Of these 2,155 patients, 42 (1.95%; 26 male and 16 female) had at least 1 ophthalmic abnormality attributable to the disease. The diagnoses in these 42 patients were mycosis fungoides in 19, clinical variants of T-cell lymphoma of the skin (most commonly, peripheral T-cell lymphoma) in 11, and Sezary syndrome in 12. Cicatricial eyelid ectropion was the most common finding, affecting 17 (40.4%) of the 42 patients. Thirty-seven patients had findings that, although probably not a direct consequence of cutaneous T-cell lymphoma, have been cataloged in previous studies. CONCLUSION: Although ophthalmic abnormalities in patients with cutaneous T-cell lymphoma are relatively uncommon, the manifestations of the disease are diverse and frequently difficult to treat.  (+info)

Depressed IL-12-mediated signal transduction in T cells from patients with Sezary syndrome is associated with the absence of IL-12 receptor beta 2 mRNA and highly reduced levels of STAT4. (3/261)

Sezary syndrome (SS) is the leukemic phase of cutaneous T cell lymphoma characterized by the proliferation of clonally derived CD4+ T cells that release cytokines of the Th2 T cell phenotype (IL-4, IL-5, IL-10), whereas Th1 T cell cytokines (IL-2, IFN-gamma) are markedly depressed as is expression of IL-12, a pivotal cytokine for Th1 cell differentiation. Normal Th1 cells express both the beta 1 and beta 2 chains of the IL-12 receptor (IL-12R) and tyrosine phosphorylate STAT4 in response to IL-12. Th2 T cells express only the IL-12R beta 1 and thus do not tyrosine phosphorylate STAT4 in response to IL-12. To determine whether SS cells are Th2-like at the level of IL-12 signal transduction, we analyzed RNA from seven patients for the presence of message for the IL-12R beta 1 and beta 2 genes using RNase protection assays and assessed whether IL-12 induced tyrosine-phosphorylation of STAT4 by immunoblotting. In PBL from six of seven SS patients tested, beta 2 message was expressed at low to undetectable levels and its expression could not be stimulated by either IFN-alpha or IFN- gamma, which stimulated beta 2 expression in control PBL. The absence of beta 2 expression is further supportive evidence for the Th2 lineage of SS cells. However, unlike normal Th2 cells, SS cells also showed severely reduced levels of STAT4, suggesting that they have a depressed response to any inducer of the STAT4 signal transduction pathway, including IFN-alpha. This is the first observation linking STAT4 gene expression with a human disease and suggests that dysregulation of STAT4 expression may be significant to the development and/or progression of SS.  (+info)

Skewed expression of activation, differentiation and homing-related antigens in circulating cells from patients with cutaneous T cell lymphoma associated with CD7- T helper lymphocytes expansion. (4/261)

Mycosis fungoides and Sezary syndrome represent the most frequent forms of cutaneous T cell lymphoma. Both are characterized by skin infiltrating and/or circulating malignant cells displaying a CD4+CD7- phenotype in the majority of cases. Because an expansion of CD4+CD7- cells may also be found in inflammatory dermatoses or in the aging process, we evaluated, by flow cytometry, the relationship between CD7 expression and the distribution of differentiation/activation or homing antigens on peripheral blood lymphocytes from 36 cutaneous T cell lymphoma patients and from healthy donors. CD4+CD7- cells were increased in all patients with cutaneous T cell lymphoma. As a consequence, the CD7+/- ratio was reduced in stage I-II mycosis fungoides (3.96 vs 6.55 in healthy donors), and inverted in stage III-IV MF and Sezary syndrome (0.28 and 0.12 respectively). In the late stage of disease, the CD7+/- inverted ratio was strictly related to the expression of CD15s, CD60, and CD45R0, and the lack of expression of CD26 and CD49d. Interestingly, in leukemic patients, this phenotype was also associated with peculiar morphologic (large size) or phenotypical (CD3dim expression) characteristics. Furthermore, a progressive reduction of circulating CD8+ cells was also seen throughout all stages of disease. The presence of these populations in cutaneous T cell lymphoma at late phases of disease and Sezary syndrome suggests that all of these molecules may play an important part in the activation pathway and skin homing of circulating T cells in lymphoproliferative disorders. Therefore, this may constitute a distinctive feature in cutaneous T cell lymphoma patients with more aggressive characteristics.  (+info)

Sezary T-cell activating factor is a Chlamydia pneumoniae-associated protein. (5/261)

We previously identified a protein that was stimulatory for malignant Sezary T cells, termed Sezary T-cell activating factor (SAF). However, the identity of this protein has not been fully elucidated, nor has it's role been determined in the pathogenesis of cutaneous T-cell lymphoma (CTCL). The basis for epidermotropism and proliferation of malignant cells in the skin of patients with CTCL is unknown. Using a monoclonal antibody inhibitory for SAF activity, we demonstrated that SAF is present in the skin of 16 of 27 samples from patients with mycosis fungoides, the predominant form of CTCL. In this report, the SAF determinant is demonstrated to be associated with Chlamydia pneumoniae bacteria by immunohistochemistry, immunoelectron microscopy, and culture analysis. Reactivity of antibodies against an outer membrane protein of C. pneumoniae or against the lipopolysaccharide of Chlamydiae spp. demonstrated that these determinants are coexpressed in 90% of the SAF-positive samples. We confirmed the presence of C. pneumoniae DNA and RNA in the skin by PCR and reverse transcription-PCR and by sequence analysis of the PCR products. The expression of the C. pneumoniae antigens and SAF appears to be associated with active disease in that C. pneumoniae antigens were absent or greatly diminished in the skin of three patients examined after Psoralen and long-wave UVA radiation treatment. Our results suggest that SAF is a Chlamydia-associated protein and that further investigation is warranted to determine whether SAF and C. pneumoniae play a role in the pathogenesis of CTCL.  (+info)

Large cell transformation of Sezary syndrome. A conventional and molecular cytogenetic study. (6/261)

Hyperdiploidy sometimes is found in mycosis fungoides-Sezary syndrome, but its diagnostic significance remains undefined. We report an unusual case of Sezary syndrome manifesting with leukemic large cell transformation. Conventional karyotypic analysis showed the presence of a near-tetraploid neoplastic clone. With dual-color cytometric analysis, we showed that the large Sezary cells were near-tetraploid with a DNA index of 1.86, thereby demonstrating a direct relationship between cell size and ploidy. Comparative genomic hybridization further showed chromosomal imbalances that were not revealed on conventional karyotyping. Our findings suggest that hyperdiploidy may be a marker of large cell transformation, so that when this karyotypic abnormality is found in mycosis fungoides-Sezary syndrome, a search for such a complication is indicated.  (+info)

Fine-needle aspiration biopsy in the evaluation of lymphadenopathy associated with cutaneous T-cell lymphoma (mycosis fungoides/Sezary syndrome). (7/261)

We studied the role of fine-needle aspiration (FNA) in the evaluation of lymphadenopathy associated with cutaneous T-cell lymphoma (CTCL) in 11 patients with lymphadenopathy and compared findings with corresponding histologic material. Molecular genetic analysis for T-cell clonality by polymerase chain reaction (PCR) was performed on all aspirates. Immunophenotyping was successful in 4 of 7 cases in which flow cytometry was attempted from the aspirated material. Cytologic evaluation of FNA samples correlated strongly with histologic rating of involvement based on numbers of atypical cerebriform lymphocytes in the nodal specimen. Of 7 nodal specimens with scattered or small groups of atypical cells in the background of dermatopathic lymphadenopathy (LN1-2), the cytologic diagnosis was interpreted as reactive in all instances. Of 4 specimens with highly suspect (LN3) or definite histologic involvement (LN4), the cytologic diagnosis was likewise suspect or malignant. The correlation between molecular genetic studies on FNA samples and studies on tissue was not significant; in 2 cases, a T-cell clone was detected in the nodal tissue sample but not in the FNA sample, suggesting undersampling. A T-cell clone was detected by PCR in 5 of 7 nodal specimens judged reactive by FNA biopsy or histologic assessment. FNA for cytologic and molecular genetic analysis is a useful method to evaluate lymphadenopathy associated with CTCL and may obviate the need for surgical biopsy.  (+info)

Diminished CD3 expression is useful for detecting and enumerating Sezary cells. (8/261)

We evaluated 44 peripheral blood, bone marrow, and lymph node specimens from 23 patients with the clinical diagnosis or suspicion of cutaneous T-cell lymphoma by 4-color flow cytometry for the presence of altered CD3 expression and compared the results with light microscopic evidence of involvement by mycosis fungoides (MF) or Sezary syndrome (SS). In 19 specimens (15 peripheral blood, 3 lymph node, 1 bone marrow) from 6 patients, we noted reduced CD3 expression compared with normal T lymphocytes. Diminished CD3 expression correlated with morphologic evidence of MF/SS cells in all cases, although the enumeration of MF/SS cells differed. Comparison of the enumeration of MF/SS cells in the peripheral blood by CD3+CD7- phenotyping with CD3dim phenotyping suggested that CD3dim phenotyping was the most accurate method for counting MF/SS cells. Therefore, diminished CD3 expression as shown by flow cytometry may be particularly useful for detecting and enumerating MF/SS cells in hematopoietic tissue of patients with cutaneous T-cell lymphoma.  (+info)

Sezary Syndrome is a rare and aggressive form of cutaneous T-cell lymphoma (CTCL), a type of cancer that involves the skin's immune system. It is characterized by the presence of malignant T-lymphocytes, known as Sezary cells, in the blood, skin, and lymph nodes.

Sezary cells are typically found in large numbers in the peripheral blood, and they have a distinctive appearance with convoluted or "cerebriform" nuclei. These cells can infiltrate the skin, leading to erythroderma (a widespread redness and scaling of the skin), pruritus (severe itching), alopecia (hair loss), and lymphadenopathy (swelling of the lymph nodes).

Sezary Syndrome is often treatment-resistant, and its prognosis is generally poor. Treatment options may include chemotherapy, radiation therapy, photopheresis, immunotherapy, and stem cell transplantation.

Mycosis fungoides is the most common type of cutaneous T-cell lymphoma (CTCL), a rare cancer that affects the skin's immune system. It is characterized by the infiltration of malignant CD4+ T-lymphocytes into the skin, leading to the formation of patches, plaques, and tumors. The disease typically progresses slowly over many years, often starting with scaly, itchy rashes that can be mistaken for eczema or psoriasis. As the disease advances, tumors may form, and the lymphoma may spread to other organs, such as the lymph nodes, lungs, or spleen. Mycosis fungoides is not contagious and cannot be spread from person to person. The exact cause of mycosis fungoides is unknown, but it is thought to result from a combination of genetic, environmental, and immune system factors.

Cutaneous T-cell lymphoma (CTCL) is a type of cancer that affects T-cells, a specific group of white blood cells called lymphocytes. These cells play a crucial role in the body's immune system and help protect against infection and disease. In CTCL, the T-cells become malignant and accumulate in the skin, leading to various skin symptoms and lesions.

CTCL is a subtype of non-Hodgkin lymphoma (NHL), which refers to a group of cancers that originate from lymphocytes. Within NHL, CTCL is categorized as a type of extranodal lymphoma since it primarily involves organs or tissues outside the lymphatic system, in this case, the skin.

The two most common subtypes of CTCL are mycosis fungoides and Sézary syndrome:

1. Mycosis fungoides (MF): This is the more prevalent form of CTCL, characterized by patches, plaques, or tumors on the skin. The lesions may be scaly, itchy, or change in size, shape, and color over time. MF usually progresses slowly, with early-stage disease often confined to the skin for several years before spreading to lymph nodes or other organs.
2. Sézary syndrome (SS): This is a more aggressive form of CTCL that involves not only the skin but also the blood and lymph nodes. SS is characterized by the presence of malignant T-cells, known as Sézary cells, in the peripheral blood. Patients with SS typically have generalized erythroderma (reddening and scaling of the entire body), pruritus (severe itching), lymphadenopathy (swollen lymph nodes), and alopecia (hair loss).

The diagnosis of CTCL usually involves a combination of clinical examination, skin biopsy, and immunophenotyping to identify the malignant T-cells. Treatment options depend on the stage and subtype of the disease and may include topical therapies, phototherapy, systemic medications, or targeted therapies.

Exfoliative dermatitis is a severe form of widespread inflammation of the skin (dermatitis), characterized by widespread scaling and redness, leading to the shedding of large sheets of skin. It can be caused by various factors such as drug reactions, underlying medical conditions (like lymphoma or leukemia), or extensive eczema. Treatment typically involves identifying and removing the cause, along with supportive care, such as moisturizers and medications to control inflammation and itching. In severe cases, hospitalization may be necessary for close monitoring and management of fluid and electrolyte balance.

Skin neoplasms refer to abnormal growths or tumors in the skin that can be benign (non-cancerous) or malignant (cancerous). They result from uncontrolled multiplication of skin cells, which can form various types of lesions. These growths may appear as lumps, bumps, sores, patches, or discolored areas on the skin.

Benign skin neoplasms include conditions such as moles, warts, and seborrheic keratoses, while malignant skin neoplasms are primarily classified into melanoma, squamous cell carcinoma, and basal cell carcinoma. These three types of cancerous skin growths are collectively known as non-melanoma skin cancers (NMSCs). Melanoma is the most aggressive and dangerous form of skin cancer, while NMSCs tend to be less invasive but more common.

It's essential to monitor any changes in existing skin lesions or the appearance of new growths and consult a healthcare professional for proper evaluation and treatment if needed.

A syndrome, in medical terms, is a set of symptoms that collectively indicate or characterize a disease, disorder, or underlying pathological process. It's essentially a collection of signs and/or symptoms that frequently occur together and can suggest a particular cause or condition, even though the exact physiological mechanisms might not be fully understood.

For example, Down syndrome is characterized by specific physical features, cognitive delays, and other developmental issues resulting from an extra copy of chromosome 21. Similarly, metabolic syndromes like diabetes mellitus type 2 involve a group of risk factors such as obesity, high blood pressure, high blood sugar, and abnormal cholesterol or triglyceride levels that collectively increase the risk of heart disease, stroke, and diabetes.

It's important to note that a syndrome is not a specific diagnosis; rather, it's a pattern of symptoms that can help guide further diagnostic evaluation and management.

KIR3DL2 (Killer-cell Immunoglobulin-like Receptor 3DL2) is a type of receptor found on the surface of natural killer (NK) cells, which are a type of white blood cell in the human body's immune system. KIR3DL2 belongs to a family of receptors called KIRs (Killer-cell Immunoglobulin-like Receptors) that help NK cells recognize and respond to infected or abnormal cells.

KIR3DL2 is a inhibitory receptor, which means it can transmit a negative signal that dampens the NK cell's activation and prevents it from attacking normal, healthy cells. Specifically, KIR3DL2 recognizes and binds to HLA-A3 and HLA-A11 molecules, which are found on the surface of many human cells. When KIR3DL2 binds to these HLA molecules, it sends a signal that inhibits NK cell activation and helps prevent an immune response against normal cells.

Abnormalities in KIR3DL2 have been associated with various diseases, including certain types of cancer and autoimmune disorders. For example, some studies suggest that changes in KIR3DL2 expression or function may contribute to the development of certain leukemias and lymphomas. Additionally, variations in KIR3DL2 genes have been linked to susceptibility to autoimmune diseases such as rheumatoid arthritis and multiple sclerosis.

CD7 is a type of protein found on the surface of certain cells in the human body, including some immune cells like T-cells and natural killer cells. It is a type of antigen that can be recognized by other immune cells and their receptors, and it plays a role in the regulation of the immune response.

CD7 antigens are often used as targets for immunotherapy in certain types of cancer, as they are overexpressed on the surface of some cancer cells. For example, anti-CD7 monoclonal antibodies have been developed to target and kill CD7-positive cancer cells, or to deliver drugs or radiation directly to those cells.

It's important to note that while CD7 is a well-established target for immunotherapy in certain types of cancer, it is not a specific disease or condition itself. Rather, it is a molecular marker that can be used to identify and target certain types of cells in the body.

KIR2DL2 (Killer-cell Immunoglobulin-like Receptor 2DL2) is a type of receptor found on the surface of natural killer (NK) cells, which are a type of white blood cell in the human body's immune system. KIR2DL2 belongs to the family of KIR receptors that recognize and interact with Human Leukocyte Antigens (HLAs) expressed on the surface of other cells.

More specifically, KIR2DL2 is an inhibitory receptor that recognizes HLA-C group 2 molecules, which are a type of class I major histocompatibility complex (MHC) molecule. When KIR2DL2 binds to its ligand, it sends a negative signal that dampens the NK cell's activation and prevents it from attacking and killing the target cell.

Therefore, KIR2DL2 plays an essential role in regulating NK cell activity and maintaining immune tolerance by preventing the destruction of healthy cells. Variations in KIR genes, including KIR2DL2, have been associated with susceptibility to various diseases, including autoimmune disorders, viral infections, and cancer.

Extracorporeal photopheresis (ECP) is a medical treatment that involves the separation of peripheral blood mononuclear cells, which are then exposed to photoactivation. This process is repeated in multiple sessions and is used primarily in the management of cutaneous T-cell lymphoma (CTCL), as well as for the prevention of organ rejection in transplant patients. The exposure to photoactivation leads to the induction of apoptosis, or programmed cell death, in the affected cells, which can help to reduce inflammation and modulate the immune response. ECP is typically administered in an outpatient setting and has been shown to be a safe and effective treatment option for many patients with CTCL.

Dipeptidyl peptidase 4 (DPP-4) is a serine protease enzyme that is widely distributed in various tissues and organs, including the kidney, liver, intestines, and immune cells. It plays a crucial role in regulating several biological processes, such as glucose metabolism, immune function, and cell signaling.

In terms of glucose metabolism, DPP-4 is responsible for breaking down incretin hormones, including glucagon-like peptide-1 (GLP-1) and glucose-dependent insulinotropic polypeptide (GIP), which are released from the gut in response to food intake. These hormones stimulate insulin secretion from pancreatic beta cells, suppress glucagon release, and promote satiety, thereby helping to regulate blood sugar levels. By degrading GLP-1 and GIP, DPP-4 reduces their activity and contributes to the development of type 2 diabetes.

DPP-4 inhibitors are a class of drugs used to treat type 2 diabetes by blocking the action of DPP-4 and increasing incretin hormone levels, leading to improved insulin secretion and glucose control.

Lysosome-Associated Membrane Protein 3 (LAMP-3), also known as CD68, is a type I transmembrane glycoprotein that is primarily expressed on the lysosomal membranes of various cell types. It belongs to the LAMP family of proteins, which includes LAMP-1 and LAMP-2, that play crucial roles in maintaining the integrity and function of lysosomes.

LAMP-3 contains several structural features such as a large heavily glycosylated luminal domain, a transmembrane region, and a short cytoplasmic tail. The luminal domain is highly resistant to proteolysis due to its extensive glycosylation, which helps protect the lysosomal membrane from degradation by hydrolytic enzymes within the lysosome.

LAMP-3 has been found to be involved in various cellular processes, including antigen presentation, autophagy, and lysosomal exocytosis. It is also considered a marker for late endosomes/lysosomes and is often used as a tool to study the biology of these organelles.

In summary, Lysosome-Associated Membrane Protein 3 (LAMP-3) is a glycoprotein localized on lysosomal membranes that plays essential roles in maintaining lysosomal integrity and function while being involved in several cellular processes.

Lymphatic diseases refer to a group of conditions that affect the lymphatic system, which is an important part of the immune and circulatory systems. The lymphatic system consists of a network of vessels, organs, and tissues that help to transport lymph fluid throughout the body, fight infection, and remove waste products.

Lymphatic diseases can be caused by various factors, including genetics, infections, cancer, and autoimmune disorders. Some common types of lymphatic diseases include:

1. Lymphedema: A condition that causes swelling in the arms or legs due to a blockage or damage in the lymphatic vessels.
2. Lymphoma: A type of cancer that affects the lymphatic system, including Hodgkin's and non-Hodgkin's lymphoma.
3. Infections: Certain bacterial and viral infections can affect the lymphatic system, such as tuberculosis, cat-scratch disease, and HIV/AIDS.
4. Autoimmune disorders: Conditions such as rheumatoid arthritis, lupus, and scleroderma can cause inflammation and damage to the lymphatic system.
5. Congenital abnormalities: Some people are born with abnormalities in their lymphatic system, such as malformations or missing lymph nodes.

Symptoms of lymphatic diseases may vary depending on the specific condition and its severity. Treatment options may include medication, physical therapy, surgery, or radiation therapy. It is important to seek medical attention if you experience symptoms of a lymphatic disease, as early diagnosis and treatment can improve outcomes.

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.

PUVA therapy is a type of treatment that uses both medication and light to treat certain skin conditions, such as psoriasis, eczema, and cutaneous T-cell lymphoma. The name "PUVA" stands for Psoralen + UVA, which refers to the two main components of the therapy:

1. Psoralen: This is a medication that makes the skin more sensitive to light. It can be taken orally or applied directly to the skin in the form of a cream or bath.
2. UVA: This stands for Ultraviolet A, which is a type of light that is part of the natural sunlight spectrum. In PUVA therapy, the skin is exposed to a controlled dose of UVA light in a special booth or room.

When psoralen is introduced into the body, it absorbs into the skin and makes it more sensitive to UVA light. When the skin is then exposed to UVA light, it triggers a chemical reaction that slows down the growth of affected skin cells. This helps to reduce inflammation, scaling, and other symptoms associated with the skin condition being treated.

It's important to note that PUVA therapy can have side effects, including sunburn, itching, redness, and an increased risk of skin cancer over time. As such, it is typically used as a second-line treatment when other therapies have not been effective, and it is closely monitored by a healthcare professional to ensure its safe and effective use.

Pentostatin is a medication used in the treatment of certain types of cancer, including hairy cell leukemia and certain T-cell lymphomas. It is a type of drug called a purine nucleoside analog, which works by interfering with the production of DNA and RNA, the genetic material found in cells. This can help to stop the growth and multiplication of cancer cells.

Pentostatin is given intravenously (through an IV) in a healthcare setting, such as a hospital or clinic. It is usually administered on a schedule of every other week. Common side effects of pentostatin include nausea, vomiting, diarrhea, and loss of appetite. It can also cause more serious side effects, such as low blood cell counts, infections, and liver problems.

It's important to note that this is a medical definition of the drug and its use, and it should not be used as a substitute for professional medical advice. If you have any questions about pentostatin or your treatment, it is best to speak with your healthcare provider.

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.

Pruritus is a medical term derived from Latin, in which "prurire" means "to itch." It refers to an unpleasant sensation on the skin that provokes the desire or reflex to scratch. This can be caused by various factors, such as skin conditions (e.g., dryness, eczema, psoriasis), systemic diseases (e.g., liver disease, kidney failure), nerve disorders, psychological conditions, or reactions to certain medications.

Pruritus can significantly affect a person's quality of life, leading to sleep disturbances, anxiety, and depression. Proper identification and management of the underlying cause are essential for effective treatment.

In medical terms, the skin is the largest organ of the human body. It consists of two main layers: the epidermis (outer layer) and dermis (inner layer), as well as accessory structures like hair follicles, sweat glands, and oil glands. The skin plays a crucial role in protecting us from external factors such as bacteria, viruses, and environmental hazards, while also regulating body temperature and enabling the sense of touch.

Beta-chain gene rearrangement in the T-cell antigen receptor (TCR) refers to the genetic process that occurs during the development of T cells, a type of white blood cell crucial for adaptive immunity. The TCR is a heterodimeric protein complex expressed on the surface of T cells, responsible for recognizing and binding to specific peptide antigens presented in the context of major histocompatibility complex (MHC) molecules.

The beta-chain of the TCR is encoded by a set of gene segments called V (variable), D (diversity), J (joining), and C (constant) segments, located on chromosome 7 in humans. During T-cell development in the thymus, the following rearrangement events occur:

1. A random selection and recombination of a V, D, and J segment take place, forming a variable region exon that encodes the antigen-binding site of the beta-chain. This process introduces nucleotide insertions or deletions at the junctions between these segments, further increasing diversity.
2. The rearranged VDJ segment then combines with a C segment through RNA splicing to form a continuous mRNA sequence that encodes the complete beta-chain protein.
3. The resulting beta-chain pairs with an alpha-chain (encoded by similar gene segments on chromosome 14) to create a functional TCR heterodimer, which is then expressed on the T-cell surface.

This gene rearrangement process allows for the generation of a vast array of unique TCRs capable of recognizing various peptide antigens, ensuring broad coverage against potential pathogens and tumor cells.

T-cell lymphoma is a type of cancer that affects the T-cells, which are a specific type of white blood cell responsible for immune function. These lymphomas develop from mature T-cells and can be classified into various subtypes based on their clinical and pathological features.

T-cell lymphomas can arise in many different organs, including the lymph nodes, skin, and other soft tissues. They often present with symptoms such as enlarged lymph nodes, fever, night sweats, and weight loss. The diagnosis of T-cell lymphoma typically involves a biopsy of the affected tissue, followed by immunophenotyping and genetic analysis to determine the specific subtype.

Treatment for T-cell lymphomas may include chemotherapy, radiation therapy, immunotherapy, or stem cell transplantation, depending on the stage and aggressiveness of the disease. The prognosis for T-cell lymphoma varies widely depending on the subtype and individual patient factors.

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.

Circulating neoplastic cells (CNCs) are defined as malignant cancer cells that have detached from the primary tumor site and are found circulating in the peripheral blood. These cells have undergone genetic and epigenetic changes, leading to uncontrolled cell growth and division, and can form new tumors at distant sites in the body, a process known as metastasis.

The presence of CNCs has been shown to be a prognostic factor for poor outcomes in various types of cancer, including breast, colon, and prostate cancer. The detection and characterization of CNCs can provide valuable information about the tumor's biology, aggressiveness, and response to therapy, allowing for more personalized treatment approaches.

However, the detection of CNCs is challenging due to their rarity in the bloodstream, with only a few cells present among billions of normal blood cells. Therefore, highly sensitive methods such as flow cytometry, polymerase chain reaction (PCR), and next-generation sequencing are used for their identification and quantification.

"Sezary syndrome". Retrieved 2008-02-15. Fragkos, Konstantinos C. (2017). "Plantar keratoderma of Sézary syndrome". Clinical ... Sezary Syndrome lymphoma information (CS1 errors: periodical ignored, Articles with short description, Short description is ... Sézary's cell at Who Named It? Cuneo, A; Castoldi, GL (2005). "Mycosis fungoidses/Sezary's syndrome". Atlas Genet Cytogenet ... doi:10.1007/978-3-319-14729-1_8. ISBN 978-3-319-14728-4. "Sezary syndrome". genetic and rare diseases information center. ...
September 2007). "Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the ... "Mycosis Fungoides/Sezary Syndrome". DynaMed. Retrieved 2021-11-29. Ottevanger R, van Beugen S, Evers AW, Willemze R, Vermeer MH ... Mycosis fungoides, also known as Alibert-Bazin syndrome or granuloma fungoides, is the most common form of cutaneous T-cell ... Mycosis fungoides (MF) and Sézary syndrome (SS) are related conditions, with the same type of cancer t-lymphocytes, that ...
Castoldi G, Cuneo A (May 2005). "Mycosis fungoides/Sezary's syndrome". Atlas of Genetics and Cytogenetics in Oncology and ... Vorinostat has also been used to treat Sézary syndrome, another type of lymphoma closely related to CTCL. A recent study ... It has given encouraging results in a phase II trial for myelodysplastic syndromes in combination with idarubicin and ... with idarubicin and cytarabine for patients with newly diagnosed acute myelogenous leukemia or myelodysplastic syndrome". ...
Sezary syndrome) abnormalities, each presenting distinct challenges in diagnosis and management. A low normal to low absolute ... HIV ultimately progresses to acquired immune deficiency syndrome (AIDS). The effects of other viruses or lymphocyte disorders ...
Ortonne N, Bagot M, Bensussan A (2006). "[KIR3DL2: a new step for the management of patients with Sezary syndrome]" (PDF). Med ...
March 2002). "Frequent abnormalities of the p15 and p16 genes in mycosis fungoides and sezary syndrome". The Journal of ...
Also, a lack of CD7 expression could insinuate mycosis fungoides (MF) or Sezary syndrome (SS). GRCh38: Ensembl release 89: ... predictors of outcome in the treatment of Sézary syndrome and erythrodermic mycosis fungoides with extracorporeal photopheresis ... "Differences in blast immunophenotypes among disease types in myelodysplastic syndromes: a multicenter validation study". ...
"Sezary syndrome cells unlike normal circulating T lymphocytes fail to migrate following engagement of NT1 receptor". The ...
The disorder is usually indolent but infrequently progresses to T-cell lymphoma or Sezary syndrome. Accumulation of partial ... Congenital disorders Hyperimmunoglobulin E syndrome Omenn syndrome Familial eosinophilia Eosinophilia-myalgia syndrome ... Hypereosinophilic Syndrome research in UK Archived 2018-08-05 at the Wayback Machine Hypereosinophilic Syndrome on patient.info ... Wiskott-Aldrich syndrome (defective WAS gene), IPEX syndrome (defective IPEX gene), CD40 gene defect, and autoimmune ...
... sezary syndrome MeSH C04.557.386.480.750.825 - lymphoma, t-cell, peripheral MeSH C04.557.386.480.875 - lymphoma, ... denys-drash syndrome MeSH C04.557.435.595.950 - wagr syndrome MeSH C04.557.435.600 - nephroma, mesoblastic MeSH C04.557.435.675 ... denys-drash syndrome MeSH C04.588.945.947.535.585.950 - wagr syndrome MeSH C04.588.945.947.535.790 - nephroma, mesoblastic MeSH ... denys-drash syndrome MeSH C04.700.635.950 - wagr syndrome MeSH C04.700.645.650 - neurofibromatosis 1 MeSH C04.700.645.655 - ...
... zone lymphoma Nodal marginal zone B-cell lymphoma M9700/3 Mycosis fungoides Pagetoid reticulosis M9701/3 Sezary syndrome Sezary ... M9895/3 Acute myeloid leukemia multilineage dysplasia AML with/without prior myelodysplastic syndrome M9896/3 AML with t(8;21)( ... hypereosinophilic syndrome M9970/1 Lymphoproliferative disease/disorder, NOS Post-transplant lymphoproliferative disorder, ... M9920/3 Acute myeloid leukemia and myelodysplastic syndrome, therapy related, NOS Therapy-related acute myeloid leukemia, ...
Churg-Strauss syndrome) Acute and chronic eosinophilic pneumonia Mycosis fungoides (MF) Sezary syndrome (SS) Lymphocytic ... Maculopapular exanthema Stevens-Johnson syndrome/toxic epidermal necrolysis (Non-)episodic angioedema with eosinophilia ...
... sezary syndrome MeSH C15.604.515.880 - tumor lysis syndrome MeSH C15.604.515.925 - waldenstrom macroglobulinemia MeSH C15.604. ... sezary syndrome MeSH C15.604.515.569.480.750.825 - lymphoma, t-cell, peripheral MeSH C15.604.515.569.480.875 - lymphoma, ... chediak-higashi syndrome MeSH C15.378.553.774.535 - granulomatous disease, chronic MeSH C15.378.553.774.600 - job's syndrome ... waterhouse-friderichsen syndrome MeSH C15.378.463.960 - wiskott-aldrich syndrome MeSH C15.378.553.231 - eosinophilia MeSH ...
"Sézary's disease" (Sézary's syndrome): A form of cutaneous T-cell lymphoma. A variant of mycosis fungoides. Sur la pathogénie ... Biography of Albert Sézary at Who Named It Albert Sezary Archived 2016-04-26 at the Wayback Machine BIU Santé, Paris Quelques ...
Mycosis Fungoides/Sezary syndrome. In addition to the NCCN guidelines, the European Organization for Research and Treatment of ... What is the role of participation in a clinical trial in the treatment of mycosis fungoides/Sezary syndrome (MF/SS) non-Hodgkin ... What are the NCCN recommendations for the diagnostic workup of mycosis fungoides/Sezary syndrome (MF/SS) in cutaneous T-cell ... What is the revised TNMB classification for mycosis fungoides/Sezary syndrome (MF/SS) in cutaneous T-cell lymphoma (CTCL) non- ...
Mycosis Fungoides/Sezary syndrome. In addition to the NCCN guidelines, the European Organization for Research and Treatment of ... What is the role of participation in a clinical trial in the treatment of mycosis fungoides/Sezary syndrome (MF/SS) non-Hodgkin ... What are the NCCN recommendations for the diagnostic workup of mycosis fungoides/Sezary syndrome (MF/SS) in cutaneous T-cell ... What is the revised TNMB classification for mycosis fungoides/Sezary syndrome (MF/SS) in cutaneous T-cell lymphoma (CTCL) non- ...
Low SATB1 expression promotes IL-5 and IL-9 expression in Sezary Syndrome.. ...
RATIONALE: Lenalidomide may stop the growth of mycosis fungoides/Sezary syndrome by blocking blood flow to the cancer. PURPOSE ... Lenalidomide in Treating Patients With Relapsed Mycosis Fungoides/Sezary Syndrome. Not Recruiting ... Histologically confirmed mycosis fungoides/Sézary syndrome - Stage IA-IVB disease - Must have failed ≥ 1 prior topical ...
Erythrophagocytic OKT4+ lymphocytes with suppressor activity in Sezary syndrome. / Shohat, B.; David, M.; Alkalay, J. et al. In ... Shohat B, David M, Alkalay J, Sandbank M. Erythrophagocytic OKT4+ lymphocytes with suppressor activity in Sezary syndrome. ... Erythrophagocytic OKT4+ lymphocytes with suppressor activity in Sezary syndrome. In: Cancer Journal. 1989 ; Vol. 2, No. 8. pp. ... In 1986 the clinical picture was compatible with Sezary syndrome. Ninety-nine percent of her peripheral blood lymphocytes were ...
"Sezary syndrome". Retrieved 2008-02-15. Fragkos, Konstantinos C. (2017). "Plantar keratoderma of Sézary syndrome". Clinical ... Sezary Syndrome lymphoma information (CS1 errors: periodical ignored, Articles with short description, Short description is ... Sézarys cell at Who Named It? Cuneo, A; Castoldi, GL (2005). "Mycosis fungoidses/Sezarys syndrome". Atlas Genet Cytogenet ... doi:10.1007/978-3-319-14729-1_8. ISBN 978-3-319-14728-4. "Sezary syndrome". genetic and rare diseases information center. ...
Sezary syndrome and mycosis fungoides arise from distinct T-cell subsets: a biologic rationale for their distinct clinical ... Mycosis fungoides and Sezary syndrome. Lancet. 2008 Mar 15;371(9616):945-57. doi: 10.1016/S0140-6736(08)60420-1. Citation on ... Mycosis fungoides and Sezary syndrome: clinical, histopathological and immunohistochemical review and update. An Bras Dermatol ... Oncogenomic analysis of mycosis fungoides reveals major differences with Sezary syndrome. Blood. 2009 Jan 1;113(1):127-36. doi ...
Mycosis Fungoides/Sezary syndrome. In addition to the NCCN guidelines, the European Organization for Research and Treatment of ... What is the role of participation in a clinical trial in the treatment of mycosis fungoides/Sezary syndrome (MF/SS) non-Hodgkin ... What are the NCCN recommendations for the diagnostic workup of mycosis fungoides/Sezary syndrome (MF/SS) in cutaneous T-cell ... What is the revised TNMB classification for mycosis fungoides/Sezary syndrome (MF/SS) in cutaneous T-cell lymphoma (CTCL) non- ...
Mycosis fungoides / Sezary syndrome. *Primary cutaneous CD30-positive T cell lymphoproliferative disorders *Primary cutaneous ...
The working diagnosis was Sezary syndrome with erythroderma. He was transferred to our intensive care unit with widespread ...
Duvelisib and Nivolumab for the Treatment of Stage IIB-IVB Mycosis Fungoides and Sezary Syndrome. *. Location:. Interventional ... Duvelisib and Nivolumab for the Treatment of Stage IIB-IVB Mycosis Fungoides and Sezary Syndrome. Scottsdale/Phoenix, AZ, ... or treating with the usual approach in patients with mycosis fungoides and Sezary syndrome. ... of duvelisib in combination with nivolumab in treating patients with stage IIB-IVB mycosis fungoides and Sezary syndrome. ...
Cutaneous T-cell lymphoma (mycosis fungoides/Sezary syndrome). *Lymphomatoid papulosis and other lymphoproliferative disorders ...
Sezary Syndrome * Small Bowel Cancer * Small Intestine Cancer * Spindle Cell Hemangioma * Stomach Cancer ...
Membranous-Like Glomerulopathy with Masked IgG Kappa Deposits (MGMID) in a Patient with Sezary Syndrome. 10:00 AM - 12:00 PM. ... Membranous-Like Glomerulopathy with Masked IgG Kappa Deposits (MGMID) in a Patient with Sezary Syndrome. November 04, 2023 , 10 ... Nephrotic Syndrome with Monoclonal Gammopathy of Undetermined Significance (MGUS): A Case Report. 10:00 AM - 12:00 PM. ... Nephrotic Syndrome with Monoclonal Gammopathy of Undetermined Significance (MGUS): A Case Report. November 04, 2023 , 10:00 AM ...
Sezary syndrome is a systemic form of CTCL manifesting with diffuse erythroderma, lymphadenopathy, and circulating malignant ... Foss F: Mycosis fungoides and the Sezary syndrome. Curr Opin Oncol 16:421-428, 2004 ...
Sezary Syndrome: A Case Report and a Review of the Molecular Pathomechanism and Management. P P L Ng, C L Goh ... Clinical Report: A Case of Williams Syndrome and Klinefelter Syndrome. Le Ye Lee, Swee Chye Quek, Samuel S Chong, Arnold SC Tan ... Cushings syndrome is usually easy to recognise clinically. Determining the cause of non-iatrogenic Cushings syndrome is, on ... Hepatopulmonary Syndrome: A Rare Complication of Chronic Liver Disease in Children. F K P Yap, M M Aw, S C Quek, S H Quak ...
Sezary Syndrome: A Case Report and a Review of the Molecular Pathomechanism and Management. P P L Ng, C L Goh ... Dapsone Hypersensitivity Syndrome Masquerading as a Viral Exanthem: Three Cases and a Mini-Review. AKH Tee, HML Oh, IYJ Wee, BP ... Allopurinol Hypersensitivity Syndrome and Acute Myocardial Infarction-Two Case Reports. Y C Chan, Y K Tay, S K Ng ... Sweets Syndrome Associated with Mycobacterium chelonae and Herpes Simplex Virus Infections: A Case Report. T S C Theng, Y C ...
Sezary syndrome. HR Chandrashekhar, SR Kadam, P Madhavmurthy, Keta Pai Full text , PDF ​ ...
Sezary syndrome. United Vilnius and Riga Dermatovenereology Students Groups Meeting. Immune Deficiency and Dermatological ... Rothmund-Thomson syndrome. United Vilnius and Riga Dermatovenereology Students Groups Meeting. Rare diseases. 2020 m. ...
A cutaneous lymphoma international prognostic index (CLIPi) for mycosis fungoides and Sezary syndrome. Eur J Cancer. 2013 Sep; ... and used during follow up for CTCL patients with advanced stage mycosis fungoides/Sezary Syndrome (MF/SS) or other aggressive ... Lebowitz et al evaluated 174 elderly patients with mycosis fungoides (MF) and Sézary syndrome (SS); 76.4% were diagnosed with ... Survival, disease progression and prognostic factors in elderly patients with mycosis fungoides and Sézary syndrome: A ...
TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and Sezary syndrome: a proposal of the ... Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society ... EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome. Eur J Cancer. 2006 May. 42 (8):1014-30 ... Clinical end points and response criteria in mycosis fungoides and Sézary syndrome: a consensus statement of the International ...
Afiat TP, Zhang X, Zhang H, Ayala E, Zhang L, Sokol L. Sezary syndrome manifesting as posttransplant lymphoproliferative ...
Impact of alemtuzumab on HIV persistence in an HIV-infected individual on antiretroviral therapy with Sezary syndrome. ... Cryptococcosis-Associated Immune Reconstitution Inflammatory Syndrome Is Associated With Dysregulation of IL-7/IL-7 Receptor ... Association between plasma antibody responses and risk for Cryptococcus-associated immune reconstitution inflammatory syndrome ... Journal of Acquired Immune Deficiency Syndromes. 84, 5, p. 527-533 7 p.. Research output: Contribution to journal › Article › ...
Regarding skin lymphomas a large recent series of patients with mycosis fungoides and Sezary syndrome showed that 91% of ... "Retrospective 5-year review of 131 patients with mycosis fungoides and Sézary syndrome seen at the National Skin Centre, ...
... mycosis fungoides and Sezary syndrome) after a non-myeloablative allogeneic transplant. Approximately 50% of the patients ...
In particular, differences in the classification of cutaneous T-cell lymphomas other than mycosis fungoides, Sezary syndrome, ... Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part I: Clinical and histologic features ... Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part I. Diagnosis: clinical and histopathologic ... In particular, differences in the classification of cutaneous T-cell lymphomas other than mycosis fungoides, Sezary syn … ...
Cutaneous T cell lymphoma: Excessive activation of the mTOR pathway is found in sezary syndrome and other cutaneous T cell ... This includes Cowden syndrome, Lhermitte-Duclos syndrome and Bannayan Riley-Ruvalcaba syndrome. The mTOR inhibitor (oral ... Familial multiple discoid fibromas and Birtt-Hogg-Dube syndrome: It occurs due to mutation in folliculin gene characterised by ... Phosphatase and tensin homolog hamartoma tumour syndrome: This is an autosomal dominant group of disorders with hamartomas in ...
  • RATIONALE: Lenalidomide may stop the growth of mycosis fungoides/Sezary syndrome by blocking blood flow to the cancer. (stanford.edu)
  • PURPOSE: This phase II trial is studying how well lenalidomide works in treating patients with relapsed mycosis fungoides/Sezary syndrome. (stanford.edu)
  • The immunohistochemical features are very similar to those presented in mycosis fungoides except for the following differences: More monotonous cellular infiltrates (large, clustered atypical pagetoid cells) in Sézary syndrome Sometimes absent epidermotropism Increased lymph node involvement with infiltrates of Sézary syndrome. (wikipedia.org)
  • In one such example, HTS was used to measure the patient-specific TCR sequences in patients with cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome) after a non-myeloablative allogeneic transplant. (stanford.edu)
  • While skin cancer can manifest in various forms, this comprehensive guide will focus on four major types: Basal Cell Carcinoma, Cutaneous T-Cell Lymphoma, Mycosis Fungoides, and Sezary Syndrome. (senderrarx.com)
  • Two common subtypes of CTCL include Mycosis Fungoides and Sezary Syndrome. (senderrarx.com)
  • This phase I trial identifies the best dose, possible benefits, and/or side effects of duvelisib in combination with nivolumab in treating patients with stage IIB-IVB mycosis fungoides and Sezary syndrome. (clfoundation.org)
  • Giving duvelisib in combination with nivolumab may work better than giving each of these drugs individually, or treating with the usual approach in patients with mycosis fungoides and Sezary syndrome. (clfoundation.org)
  • Results from a multi-center phase III trial examiniing the safety and efficacy of two doses of denileukin diftitox (Ontak) in patients with biopsy-confirmed CD25 assay-positive cutaneous T-cell lymphoma (CTCL), including both mycosis fungoides and Sezary Syndrome suggest merely that this recombinant protein can outperform a placebo. (lymphomainfo.net)
  • subtypes of cutaneous T-cell lymphoma, SS is a malignant neoplasm originating the most common of which are mycosis from T lymphocytes, which involves the fungoides and Sézary syndrome (SS). (bvsalud.org)
  • Mycosis fungoides and Sézary syndrome are uncommon chronic T-cell non-Hodgkin lymphomas primarily affecting the skin and occasionally the lymph nodes. (msdmanuals.com)
  • Mycosis fungoides and Sézary syndrome are the 2 main types of cutaneous T-cell lymphomas. (msdmanuals.com)
  • Sézary disease, or Sézary syndrome, is a type of cutaneous T-cell lymphoma that was first described by Albert Sézary. (wikipedia.org)
  • KIR3DL2 is also a marker for atypical mononuclear (Sezary) cells in the blood of patients with Sezary syndrome, an erythrodermic form of cutaneous T cell lymphoma (11). (rndsystems.com)
  • Begue E, Michel L, Jean-Louis F, Bagot M, Bensussan A (2013) Promoter Hypomethylation and Expression of PLS3 in Human Sezary Lymphoma Cells. (symbiosisonlinepublishing.com)
  • In our practice, hematologic neoplasms are mainly lymphoproliferative syndromes and the most common varieties are non-Burkitt non Hodgkin lymphoma, high grade lymphomas, chronic lymphocytic lymphoma and multiple myeloma. (bvsalud.org)
  • What is Myelodysplastic Syndrome? (lymphomainfo.net)
  • The question ought to be what are myelodysplastic syndromes (MDS), since this is a group of similar blood and bone marrow diseases that. (lymphomainfo.net)
  • We studied two human lymphocyte cell lines derived from tumor lymphocytes of patients with Sezary syndrome, HuT-78 cells which express PLS3 mRNA and SeAx cells that do not, for methylation status and used a well-established inhibitor of DNA methylation, the nucleoside analog 5-aza-2-deoxycytidine (5-aza-dC), to further determine its effect on PLS3 gene expression in both cell lines. (symbiosisonlinepublishing.com)
  • A molecular feature of Sezary syndrome (SS), the leukemic variant of cutaneous T-cell lymphomas (CTCL), is the abnormal gene expression of PLS3 in 3 out of 4 patients [1-9]. (symbiosisonlinepublishing.com)
  • Il s'agissait d'une étude rétrospective descriptive d'une durée de 2 ans 3 mois allant du 1er janvier 2018 au 31 Mars 2020 portant sur 80 dossiers de malades porteurs d'hémopathies malignes et prise en charge dans le centre. (bvsalud.org)
  • In some cases, a leukemic phase called Sézary syndrome is characterized by the appearance of malignant T cells with serpentine nuclei in the peripheral blood. (msdmanuals.com)
  • Other malignant conditions such as Sezary syndrome {Horiuchi, 1985} osteogenic sarcoma {Barron, 1992} and transitional cell carcinoma of the urinary bladder {Yaniv, 1994} have been reported. (globale-dermatologie.com)
  • The working diagnosis was Sezary syndrome with erythroderma. (cdc.gov)
  • A bone marrow smear was performed, in which 'cerebriform' cells were identified, confirming the diagnosis of Sézary syndrome. (bvsalud.org)
  • Le protocole utilisé dans le traitement du myélome multiple a été le VMCD-REV à 76,92% avec pour réponse thérapeutique complète chez 6 patients, 3 réponses partielles et 4 en cours de traitement. (bvsalud.org)
  • In 1986 the clinical picture was compatible with Sezary syndrome. (tau.ac.il)
  • Initial management consisted of en una paciente con topical corticosteroids and oral antihistamines with little clinical response. (bvsalud.org)
  • A remarkable variety of endocrinologic disorders may cause virilization syndromes. (annals.edu.sg)
  • In Sézary syndrome, the skin is typically diffusely red with cracking of the palms and soles. (msdmanuals.com)
  • Low SATB1 expression promotes IL-5 and IL-9 expression in Sezary Syndrome. (nygenome.org)
  • Our results clearly revealed a quantitative relationship between the methylation status of the PLS3 CpG region and PLS3 expression in HuT-78 and SeAx Sezary cell lines. (symbiosisonlinepublishing.com)
  • Regarding the epidemiological distribu- skin biopsy of the dorsal and anterior chest tion of this syndrome, it predominates in region. (bvsalud.org)
  • In the Western population, there are around 3 cases of Sézary syndrome per 1,000,000 people. (wikipedia.org)
  • Sezary syndrome (SS) is a rare form of cutaneous T-cell lymphoma characterized by erythroderma and the presence of Sezary cells in the skin, lymph nodes, and peripheral blood. (nih.gov)
  • Sézary disease, or Sézary syndrome, is a type of cutaneous T-cell lymphoma that was first described by Albert Sézary. (wikipedia.org)
  • Sézary syndrome is an aggressive form of a type of blood cancer called cutaneous T-cell lymphoma. (medlineplus.gov)
  • Although Sézary syndrome is sometimes referred to as a variant of another cutaneous T-cell lymphoma called mycosis fungoides , these two cancers are generally considered separate conditions. (medlineplus.gov)
  • This study will investigate the safety and effectiveness of a modified donor stem cell transplantation procedure for treating advanced mycosis fungoides (MF), a lymphoma primarily affecting the skin, and Sezary syndrome (SS), a leukemic form of the disease. (nih.gov)
  • My mother passed on November 20, 2010 from T-Cell Lymphoma (sezary syndrome). (cancer.org)
  • The Mycosis Fungoides Cooperative Group has been following patients with cutaneous T-cell lymphoma, including mycosis fungoides and the Sezary syndrome variant. (johnshopkins.edu)
  • BACKGROUND: Ultraviolet light (UVL) is a long established treatment for mycosis fungoides (MF) and Sézary syndrome (SS), subtypes of cutaneous T-cell lymphoma (CTCL). (henryford.com)
  • Blood classification and blood response criteria in mycosis fungoides and Sézary syndrome using flow cytometry: recommendations from the EORTC cutaneous lymphoma task force. (cutaneouslymphoma.org)
  • Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer (EORTC) .BLOOD. (cutaneouslymphoma.org)
  • Sézary syndrome (SS) is an aggressive leukemic form of Cutaneous T-cell Lymphoma with neoplastic CD4+ T cells present in skin, lymph nodes, and blood. (molepi.nl)
  • Molecular cytogenetic analysis of cutaneous T-cell lymphomas: identification of common genetic alterations in Sézary syndrome and mycosis fungoides. (nih.gov)
  • Mycosis fungoides and Sézary syndrome are the 2 main types of cutaneous T-cell lymphomas. (msdmanuals.com)
  • The 2 most common types of this cancer are mycosis fungoides and the Sezary syndrome. (hopkinsmedicine.org)
  • [ 1 ] Sézary syndrome (SS) is a rare CTCL variant closely related to MF and has classically been described as a triad of erythroderma, generalized lymphadenopathy and Sézary cells (atypical neoplastic T lymphocytes with hyperconvoluted cerebriform nuclei) in the skin, blood, and lymph nodes. (medscape.com)
  • People with Sézary syndrome develop a red, severely itchy rash (erythroderma) that covers large portions of their body. (medlineplus.gov)
  • The working diagnosis was Sezary syndrome with erythroderma. (cdc.gov)
  • Mycosis fungoides that is evident as an erythroderma but with too few circulating lymphocytes to warrant a diagnosis of Sézary syndrome is designated erythrodermic mycosis fungoides. (medscape.com)
  • Evidence is presented for the existence of a "switch" T cell derived from a patient with mycosis fungoides/Sezary's syndrome. (silverchair.com)
  • [ 98 ] , the European Organization for Research and Treatment of Cancer (EORTC) released consensus recommendations for the treatment of mycosis fungoides/Sezary syndrome (MF/SS) in 2017. (medscape.com)
  • Mycosis fungoides and Sézary syndrome are uncommon chronic T-cell non-Hodgkin lymphomas primarily affecting the skin and occasionally the lymph nodes. (msdmanuals.com)
  • The immunohistochemical features are very similar to those presented in mycosis fungoides except for the following differences: More monotonous cellular infiltrates (large, clustered atypical pagetoid cells) in Sézary syndrome Sometimes absent epidermotropism Increased lymph node involvement with infiltrates of Sézary syndrome. (wikipedia.org)
  • People with Sézary syndrome also have enlarged lymph nodes (lymphadenopathy). (medlineplus.gov)
  • peripheral lymphadenopathy, and abnormal hyperchromatic mononuclear (cerebriform) cells in the skin, LYMPH NODES , and peripheral blood (Sezary cells). (bvsalud.org)
  • The systemic treatments used for advanced MF are also used to treat Sezary syndrome. (cancer.org)
  • On Aug. 8, 2018, the Food and Drug Administration approved mogamulizumab-kpkc (Poteligeo, Kyowa Kirin, Inc.) for adult patients with relapsed or refractory mycosis fungoides (MF) or Sézary syndrome (SS) after at least one prior systemic therapy. (fda.gov)
  • This phase II trial studies how well pembrolizumab and interferon gamma-1b work in treating patients with stage IB-IVB mycosis fungoides and Sezary syndrome that has come back (relapsed) or has not responded to previous treatment (refractory). (stanford.edu)
  • RATIONALE: Lenalidomide may stop the growth of mycosis fungoides/Sezary syndrome by blocking blood flow to the cancer. (stanford.edu)
  • T Cells in Sézary Syndrome Patients. (nih.gov)
  • Further evidence was obtained using mononuclear cells derived from a patient with immunodeficiency and hyper-IgM, a syndrome characterized by a lack of IgG and IgA secretion. (silverchair.com)
  • The addition of Trac cells to either peripheral blood mononuclear cells or non-T cells from a patient with hyper-IgM syndrome resulted in new secretion of IgG, with a concomitant decrease in IgM secretion, whereas control T cells were not effective in inducing secretion of any isotype other than IgM. (silverchair.com)
  • It is unclear whether these alterations play a role in Sézary syndrome, although the tendency to acquire chromosomal abnormalities (chromosomal instability) is a feature of many cancers. (medlineplus.gov)
  • Sézary syndrome is a rare condition, although its prevalence is unknown. (medlineplus.gov)
  • Molecular cytogenetic characterization of Sézary syndrome. (nih.gov)
  • Guidelines for phototherapy of mycosis fungoides and Sézary syndrome: " by Elise A. Olsen, Emilia Hodak et al. (henryford.com)
  • The final stage of mycosis fungoides is known as Sezary syndrome. (technavio.com)
  • Some people with Sézary syndrome are less able to control their body temperature than people without the condition. (medlineplus.gov)
  • Of which, mycosis fungoides (MF) and Sézary syndrome (SS) are two of the most common forms. (medscape.com)
  • EORTC consensus recommendations for the treatment of mycosis fungoides/Sezary syndrome. (cutaneouslymphoma.org)