II. Pathophysiology
- T-Cell Lymphoma that begins in skin
- Sezary Syndrome is leukemic form of T-Cell Lymphoma
III. Epidemiology
- Incidence: 0.42 cases per 100,000 (rare)
- Most common after age 50 years
- Gender: Twice as common in men
- Black patients are more commonly affected
IV. Symptoms
V. Signs (four stages of development)
VI. Complications
VII. Course
- Survival <3 years after tumors develop
VIII. References
- Habif (1996) Clinical Dermatology, Mosby, p. 674-5
- Pujol (2000) J Am Acad Dermatol 42:324-8 [PubMed]
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Related Studies
| Definition (CHV) | a kind of skin cancer |
| Definition (CHV) | a kind of skin cancer |
| Definition (NCI) | A peripheral (mature) T-cell lymphoma presenting in the skin with patches/plaques. It is characterized by epidermal and dermal infiltration of small to medium-sized T-cells with cerebriform nuclei. Patients with limited disease generally have an excellent prognosis. In the more advanced stages, the prognosis is poor. (WHO, 2001) |
| Definition (NCI_NCI-GLOSS) | A type of non-Hodgkin's lymphoma that first appears on the skin and can spread to the lymph nodes or other organs such as the spleen, liver, or lungs. |
| Definition (CSP) | chronic malignant T-cell lymphoma of the skin; in the late stages the lymph nodes and viscera are affected. |
| Definition (MSH) | A chronic, malignant T-cell lymphoma of the skin. In the late stages, the LYMPH NODES and viscera are affected. |
| Concepts | Neoplastic Process (T191) |
| MSH | D009182 |
| ICD9 | 202.1 |
| ICD10 | C84.0 , M9700/3, C84.00 |
| SnomedCT | 118618005, 190004005, 188628007, 190003004, 188618003, 90120004 |
| English | Mycosis Fungoides, Mycosis fungoides NOS, Mycosis fungoides of unspecified site, Mycosis fungoides-unspec. site, [M]Mycosis fungoides NOS, mycosis fungoides lymphoma, MYCOSIS FUNGOIDES, [M]Mycosis fungoides, mycosis fungoides, mycosis fungoides (diagnosis), Mycosis fungoides, unspecified site, Mycosis Fungoides [Disease/Finding], mf, Mycoses fungoides, Mycosis fungoides NOS (disorder), [M]Mycosis fungoides NOS (morphologic abnormality), [M]Mycosis fungoides (disorder), Mycosis fungoides of unspecified site (disorder), CTCL/ Mycosis fungoides, Cutaneous T-cell lymphoma/Mycosis fungoides, Mycosis fungoides, MF - Mycosis fungoides, Mycosis fungoides (clinical), Mycosis fungoides (disorder), Mycosis fungoides (morphologic abnormality), MF |
| Italian | Micosi fungoidi, Micosi fungoide NAS, Micosi fungoide |
| Dutch | mycosis fungoides NAO, mycoses fungoides, mycosis fungoides, Mycosis fungoides |
| French | Mycosis fongoïde SAI, MYCOSIS FUNGOIDE, Mycose fongoïde, Mycoses fongoïdes, Mycosis fongoïde, Maladie d'Alibert, Mycosis fungoïde |
| German | Mycosis fungoides NNB, Mycosis fungoides-Erkrankungen, Mycosis fungoides |
| Portuguese | Micose fungóide NE, MICOSE FUNGOIDE, Micose Fungoide, Micose fungóide, Micoses fungóides |
| Spanish | Micosis fungoide NEOM, micosis fungoide (clínica), micosis fungoide, SAI, micosis fungoide de localización no especificada, [M]micosis fungoide, SAI (anomalía morfológica), micosis fungoide, SAI (trastorno), [M]micosis fungoide, SAI, micosis fungoide de localización no especificada (trastorno), enfermedad de Woringer - Kolopp, micosis fungoide (anomalía morfológica), micosis fungoide (clínica) (trastorno), micosis fungoide (trastorno), micosis fungoide, Micosis fungoide, Micosis fungoides, Micosis Fungoide |
| Japanese | 菌状息肉症ＮＯＳ, 菌状息肉症, 菌状息肉腫, ｷﾝｼﾞｮｳｿｸﾆｸｼｮｳ, ｷﾝｼﾞｮｳｿｸﾆｸｼｮｳNOS, ｷﾝｼﾞｮｳｿｸﾆｸｼｭ |
| Swedish | Mycosis fungoides |
| Czech | mycosis fungoides, Mycosis fungoides, Mycoses fungoides, Mycosis fungoides NOS |
| Finnish | Mycosis fungoides |
| Russian | AMBERA-BAZENA MIKOZ, MIKOZ GRIBOVIDNYI, GRANULEMA GRIBOVIDNAIA, АМБЕРА-БАЗЕНА МИКОЗ, ГРАНУЛЕМА ГРИБОВИДНАЯ, МИКОЗ ГРИБОВИДНЫЙ |
| Korean | 균상식육종 |
| Croatian | MIKOZA, FUNGOIDNA, FUNGOIDNA MIKOZA |
| Polish | Ziarniniak grzybiasty |
| Hungarian | Mycosis fungoides, Mycoses fungoides, Mycosis fungoides k.m.n. |
| Norwegian | Mycosis fungoides |
Ontology: Sezary Syndrome (C0036920)
| Definition (MSHCZE) | Maligní lymfom z T buněk postihující primárně kůži s výskytem nádorových buněk rovněž v periferní krvi (někdy považován za generalizaci mycosis fungoides). Na kůži je svědící erytrodermie, hyperkeratóza, pigmentace, může být lymfadenopatie či splenomegalie. Objevují se Sézaryho-Lutznerovy buňky. Vyskytuje se obv. u starších osob s určitou převahou mužů, průběh bývá větš. chronický. Léčba je obdobná jako u mycosis fungoides. (cit. Velký lékařský slovník online, 2012 http://lekarske.slovniky.cz/ ) |
| Definition (NCI) | A generalized peripheral (mature) T-cell neoplasm characterized by the presence of erythroderma, lymphadenopathy, and neoplastic, cerebriform T-lymphocytes in the blood. Sezary syndrome is an aggressive disease. (WHO, 2001) |
| Definition (NCI_NCI-GLOSS) | A form of cutaneous T-cell lymphoma, a cancerous disease that affects the skin. |
| Definition (MSH) | 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). |
| Concepts | Neoplastic Process (T191) |
| MSH | D012751 |
| ICD9 | 202.2 |
| ICD10 | C84.1 , M9701/3, C84.10 |
| SnomedCT | 188638002, 188629004, 118611004, 4950009 |
| English | Sezary's syndrome, Erythroderma, Sezary, Sezary Erythroderma, Sezary Syndrome, Syndrome, Sezary, Sezary's disease NOS, Sezary's disease of unspecified site, Sezary's disease-unspec. site, Sezary's disease NOS (disorder), Sezary's disease of unspecified site (disorder), [M]Sezary's disease, Sezary syndrome (diagnosis), Lymphoma, Sezary's, Sezarys Lymphoma, Sezary Lymphoma, Sezary's Lymphoma, Sézary disease, Sézary disease, unspecified site, Sezary Syndrome [Disease/Finding], sezary syndrome, sezary's disease, sezary's syndrome, Sezary disease, unspecified site, Sézary's disease NOS, Sézary's disease NOS (disorder), Sézary's disease of unspecified site, Sézary's disease of unspecified site (disorder), CTCL / Sezary syndrome, Cutaneous T-cell lymphoma/Sezary syndrome, [M]Sézary's disease, Sézary's disease (clinical), Sézary's disease (disorder), Sézary; reticulosis, Sézary, reticulosis; Sézary, Sezary's Disease, Sezary's disease, Sezary syndrome, Sezary's disease (morphologic abnormality), Sézary's disease (morphologic abnormality), Sézary's disease, Sézary's syndrome |
| Spanish | [M]enfermedad de Sézary, Enfermedad de Sezary, Eritroderma de Sézary, (M)enfermedad de Sézary, enfermedad de Sézary de localización no especificada, enfermedad de Sézary, SAI (trastorno), enfermedad de Sézary, SAI, enfermedad de Sézary de localización no especificada (trastorno), enfermedad de Sézary (anomalía morfológica), enfermedad de Sézary (clínica) (trastorno), enfermedad de Sézary (clínica), enfermedad de Sézary (trastorno), enfermedad de Sézary, síndrome de Sézary, Síndrome de Sézary |
| Dutch | Sézary-ziekte, Sézary; reticulose, reticulose; Sézary, Szary-syndroomd, Sézary-reticulose, Sézary-syndroom, Syndroom, Sézary- |
| French | Maladie de Sezary, Réticulose de Sézary, Syndrome de Sézary, Érythrodermie de Sézary |
| German | Sezary-Syndrom, Sezary-Krankheit, Erythrodermie, Sézary, Sézary-Syndrom |
| Italian | Malattia di Sézary, Eritroderma di Sezary, Sindrome di Sezary |
| Portuguese | Síndrome de Sezary, Doença de Sezary, Eritroderma de Sézary, Síndrome de Sézary |
| Japanese | セザリー病, ｾｻﾞﾘｰﾋﾞｮｳ, ｾｻﾞﾘｰｼｮｳｺｳｸﾞﾝ, Sezary紅皮症, Sezary症候群, シーゼリィ病, シーゼリー症候群, セザリー症候群, セザール症候群, セザリー紅皮症, 紅皮症-セザリー |
| Swedish | Sezarys syndrom |
| Czech | Sézaryho syndrom, Sézaryho choroba |
| Finnish | Sézaryn oireyhtymä |
| Russian | ERITRODERMIIA SEZARI, SEZARI SINDROM, СЕЗАРИ СИНДРОМ, ЭРИТРОДЕРМИЯ СЕЗАРИ |
| Korean | 세자리병 |
| Polish | Zespół Sezary'ego, Erytrodermia Sezary'ego |
| Hungarian | Sézary-syndroma, Sézary-betegség, Sézary-szindróma |
| Norwegian | Sézarys syndrom |
