Dermatology Book

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Mycosis FungoidesAka: Cutaneous T-Cell Lymphoma, Sezary Syndrome, Sezary Cell, Sezary Erythroderma

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  1. Pathophysiology
    1. T-Cell Lymphoma that begins in skin
    2. Sezary Syndrome is leukemic form of T-Cell Lymphoma
  2. 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
  3. Symptoms
    1. Pruritus
  4. 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
  5. Complications
    1. Exfoliative dermatitis
  6. Course
    1. Survival <3 years after tumors develop
  7. References
    1. Habif (1996) Clinical Dermatology, Mosby, p. 674-5
    2. Pujol (2000) J Am Acad Dermatol 42:324

Sezary Syndrome (C0036920)

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).
Definition (NCI)A form of cutaneous T-cell lymphoma, a cancerous disease that affects the skin.
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)
ConceptsNeoplastic Process (T191)
ICD9202.2
EnglishSezary Erythroderma, Sezary Syndrome, Sezary's Disease, Sezary's disease of unspecified site, Sezary's syndrome
Spanishenfermedad de Sezary, enfermedad de Sezary de localizacion no especificada, sindrome de Sezary
Parent ConceptsOther malignant neoplasm of lymphoid and histiocytic tissue (C0153793), Lymphoproliferative Disorders (C0024314), Lymphoma, T-Cell, Cutaneous (C0079773), Leukemic Phase of Non-Hodgkin's Lymphoma (C0852709), mycosis fungoides/Sezary syndrome (C0862196), Aggressive Non-Hodgkin Lymphoma (C1332225), Sezary Syndrome (C0036920), Low grade T-cell lymphoma (C0456876), Mature (peripheral) T-cell neoplasm (C1532009), Primary cutaneous T-cell lymphoma - category (C1634515)
SourcesCOSTAR, CSP, DXP, ICD9CM, MSH, MTH, NCI, NDFRT, PDQ, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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