Mycosis Fungoides/Sézary syndrome (MF/SS)From $1Table of contentsCombined revision comparisonPlease contribute to the site. See the contributor's FAQ for more information. DefinitionSézary syndrome (SS) and Mycosis Fungoides (MF) are T-cell lymphomas whose primary manifestation is in the skin. Mycosis Fungoides, is the most common form of cutaneous T-cell lymphoma. It generally affects the skin, but may progress internally over time.The name mycosis fungoides is somewhat misleading--it loosely means "mushroom-like fungal disease". The disease, however, is not fungal infection but rather a type of non-Hodgkin's lymphoma. It was so named because the skin tumors of a severe case as having a mushroom-like appearance.
Sézary's disease (or "Sézary syndrome") is a type of cutaneous lymphoma characterized by Albert Sézary."Sézary's cells" are T-cells that have pathological quantities of mucopolysaccharides. Sézary's disease is sometimes considered a late stage of mycosis fungoides EpidemiologyIt is rare for the disease to appear before age 20, and it appears to be noticeably more common in males than females, especially over the age of 50, where the incidence of the disease (the risk per person in the population) does increase. The average age of onset is between 45 and 55 years of age for patients with patch and plaque disease only, but is over 60 for patients who present with tumours, erythroderma (red skin) or a leukemic form (the Sézary syndrome). Possible causesHTLV has thought to have been associated with both of the above cutaneous mycoses. MorphologyThe malignant cells are small to medium in size with irregular (cerebriform) nuclei. a minority of larger cells may be present (classical Sézary cells) but only in minority. (see below)
ImmunophenotypingThe demonstration of an elevated CD4/CD8 ratio, and increased proportions of CD4+, CD7- T-lymphocytes. Tumor cells express CD2, CD3, TCRb, CD5 and CD7 (-/+). Most cases are CD4+, rarely is CD8 expressed. Aberrant T-cell phenotypes are common.
Other relevant testsCytochemistry: Genetics:T-cell receptor genes are clonally rearranged in most cases. Complex karyotypes are present in many cases in advanced stages. Flow Diagnosis:MF/SS cells have a mature T-cell phenotype expressing CD3, CD4, CD45RO and often with aberrant expression of at least one pan-T cell antigen. Referenceshttp://www.ncbi.nlm.nih.gov/pubmed/10989648?dopt=Abstract Contributors to this page{{ wiki.contributors(page.path, 10) }} Sample CasesClick here for instructions on how to download the free FCS Express Reader to view and manipulate the sample cases.
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Version from 06:45, 21 Jul 2010Please contribute to the site. See the contributor's FAQ for more information. DefinitionSézary syndrome (SS) and Mycosis Fungoides (MF) are T-cell lymphomas whose primary manifestation is in the skin. Mycosis Fungoides, is the most common form of cutaneous T-cell lymphoma. It generally affects the skin, but may progress internally over time.The name mycosis fungoides is somewhat misleading--it loosely means "mushroom-like fungal disease". The disease, however, is not fungal infection but rather a type of non-Hodgkin's lymphoma. It was so named because the skin tumors of a severe case as having a mushroom-like appearance.
Sézary's disease (or "Sézary syndrome") is a type of cutaneous lymphoma characterized by Albert Sézary."Sézary's cells" are T-cells that have pathological quantities of mucopolysaccharides. Sézary's disease is sometimes considered a late stage of mycosis fungoides EpidemiologyIt is rare for the disease to appear before age 20, and it appears to be noticeably more common in males than females, especially over the age of 50, where the incidence of the disease (the risk per person in the population) does increase. The average age of onset is between 45 and 55 years of age for patients with patch and plaque disease only, but is over 60 for patients who present with tumours, erythroderma (red skin) or a leukemic form (the Sézary syndrome). Possible causesHTLV has thought to have been associated with both of the above cutaneous mycoses. MorphologyThe malignant cells are small to medium in size with irregular (cerebriform) nuclei. a minority of larger cells may be present (classical Sézary cells) but only in minority. (see below)
ImmunophenotypingThe demonstration of an elevated CD4/CD8 ratio, and increased proportions of CD4+, CD7- T-lymphocytes. Tumor cells express CD2, CD3, TCRb, CD5 and CD7 (-/+). Most cases are CD4+, rarely is CD8 expressed. Aberrant T-cell phenotypes are common.
Other relevant testsCytochemistry: Genetics:T-cell receptor genes are clonally rearranged in most cases. Complex karyotypes are present in many cases in advanced stages. Flow Diagnosis:MF/SS cells have a mature T-cell phenotype expressing CD3, CD4, CD45RO and often with aberrant expression of at least one pan-T cell antigen. Referenceshttp://www.ncbi.nlm.nih.gov/pubmed/10989648?dopt=Abstract Contributors to this page{{ wiki.contributors(page.path, 10) }} Sample CasesClick here for instructions on how to download the free FCS Express Reader to view and manipulate the sample cases.
Current versionPlease contribute to the site. See the contributor's FAQ for more information. DefinitionSézary syndrome (SS) and Mycosis Fungoides (MF) are T-cell lymphomas whose primary manifestation is in the skin. Mycosis Fungoides, is the most common form of cutaneous T-cell lymphoma. It generally affects the skin, but may progress internally over time.The name mycosis fungoides is somewhat misleading--it loosely means "mushroom-like fungal disease". The disease, however, is not fungal infection but rather a type of non-Hodgkin's lymphoma. It was so named because the skin tumors of a severe case as having a mushroom-like appearance.
Sézary's disease (or "Sézary syndrome") is a type of cutaneous lymphoma characterized by Albert Sézary."Sézary's cells" are T-cells that have pathological quantities of mucopolysaccharides. Sézary's disease is sometimes considered a late stage of mycosis fungoides EpidemiologyIt is rare for the disease to appear before age 20, and it appears to be noticeably more common in males than females, especially over the age of 50, where the incidence of the disease (the risk per person in the population) does increase. The average age of onset is between 45 and 55 years of age for patients with patch and plaque disease only, but is over 60 for patients who present with tumours, erythroderma (red skin) or a leukemic form (the Sézary syndrome). Possible causesHTLV has thought to have been associated with both of the above cutaneous mycoses. MorphologyThe malignant cells are small to medium in size with irregular (cerebriform) nuclei. a minority of larger cells may be present (classical Sézary cells) but only in minority. (see below)
ImmunophenotypingThe demonstration of an elevated CD4/CD8 ratio, and increased proportions of CD4+, CD7- T-lymphocytes. Tumor cells express CD2, CD3, TCRb, CD5 and CD7 (-/+). Most cases are CD4+, rarely is CD8 expressed. Aberrant T-cell phenotypes are common.
Other relevant testsCytochemistry: Genetics:T-cell receptor genes are clonally rearranged in most cases. Complex karyotypes are present in many cases in advanced stages. Flow Diagnosis:MF/SS cells have a mature T-cell phenotype expressing CD3, CD4, CD45RO and often with aberrant expression of at least one pan-T cell antigen. Referenceshttp://www.ncbi.nlm.nih.gov/pubmed/10989648?dopt=Abstract Contributors to this page{{ wiki.contributors(page.path, 10) }} Sample CasesClick here for instructions on how to download the free FCS Express Reader to view and manipulate the sample cases.
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