II. Pathophysiology

  1. T-Cell Lymphoma that begins in skin
  2. Sezary Syndrome is leukemic form of T-Cell Lymphoma

III. Epidemiology

  1. Incidence: 0.42 cases per 100,000 (rare)
  2. Most common after age 50 years
  3. Gender: Twice as common in men
  4. Black patients are more commonly affected

IV. Symptoms

V. Signs (four stages of development)

  1. Eczematous patches and Plaques
  2. Distributed over non-sun-exposed skin
    1. Buttocks
    2. Thighs
  3. Tumors develop later in course
    1. Associated with superficial and deep Lymphadenopathy
    2. Associated with metastases to Spleen, lung, GI tract

VI. Complications

VII. Course

  1. Survival <3 years after tumors develop

VIII. References

  1. Habif (1996) Clinical Dermatology, Mosby, p. 674-5
  2. Pujol (2000) J Am Acad Dermatol 42:324-8 [PubMed]

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Ontology: Mycosis Fungoides (C0026948)

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, キンジョウソクニクショウ, キンジョウソクニクショウNOS, キンジョウソクニクシュ
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, セザリービョウ, セザリーショウコウグン, 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