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Mycosis Fungoides and Sezary Syndrome

Mycosis Fungoides and Sezary Syndrome. Definitions. Mycosis fungoides: Extranodal Non-Hodgkins lymphoma of T-cell origin, with primary involvement of the skin Sezary Syndrome: Generalized erythroderma Lymphadenopathy Atypical T- cells (Sezary cells) in the peripheral blood. Epidemiology MF.

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Mycosis Fungoides and Sezary Syndrome

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  1. Mycosis Fungoides and Sezary Syndrome

  2. Definitions • Mycosis fungoides: • Extranodal Non-Hodgkins lymphoma of T-cell origin, with primary involvement of the skin • Sezary Syndrome: • Generalized erythroderma • Lymphadenopathy • Atypical T- cells (Sezary cells) in the peripheral blood

  3. Epidemiology MF • 3 cases/ 1,000,000/ year<1000/year US • Peak age 55-60 • Male: female 2:1 TIMycosis fungoides in the United States. Increasing incidence and descriptive epidemiology. AUWeinstock MA; Horm JW SOJAMA 1988 Jul 1;260(1):42-6.

  4. Clinical • Indolent cutaneous eruption with erythematous scaly patches or plaques, typically bathing trunk distribution • Heterogeneity in presentation

  5. Extracutaneous Disease • Progression to Extracutaneous disease correlates with extent of skin disease • Limited patch or plaque very rare • Generalized plaque 8 % • Tumorous or generalized erythroderma30-40% Hoppe RT, Kim YH,Clinical features, staging, and prognosis of mycosis fungoides and Sezary syndrome, Uptodate.com, 11/06

  6. Histology Hoppe RT, Kim YH,Clinical features, staging, and prognosis of mycosis fungoides and Sezary syndrome, Uptodate.com, 11/06

  7. Sezary Cell Hoppe RT, Kim YH,Clinical features, staging, and prognosis of mycosis fungoides and Sezary syndrome, Uptodate.com, 11/06

  8. TNMB classification system for mycosis fungoides • T1 Limited patch/plaque (< 10 percent of total skin surface) • T2 Generalized patch/plaque (>10 percent of total skin surface) • T3 Tumors • T4 Generalized erythroderma • N0 Lymph nodes clinically uninvolved • N1 Lymph nodes enlarged, histologically uninvolved (includes "reactive" and "dermatopathic" nodes) • N2 Lymph nodes clinically uninvolved but histologically involved • N3 Lymph nodes enlarged and histologically involved • M0 No visceral involvement • M1 Visceral involvement (histologically confirmed) • B0 No circulating atypical (Sezary) cells (< 5 percent of lymphocytes) • B1 Circulating atypical (Sezary) cells ( 5 percent of lymphocytes)

  9. Prognosis By Tumor Extent Mycosis fungoides and Sézary Syndrome. Semin Oncol 1999; 26:276. figure 1, page 279.

  10. Transformation to Large Cell • One study of 115 pt’s; 39 % with transfomation over 12 year follow up • Median time to transformation of 12 m

  11. Hoppe RT, Kim YH,Clinical features, staging, and prognosis of mycosis fungoides and Sezary syndrome, Uptodate.com, 11/06

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