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 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.

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

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

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

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

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)

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

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 Transformation of mycosis fungoides/Sezary syndrome: clinical characteristics and prognosis. Diamandidou E; Colome-Grimmer M; Fayad L; Duvic M; Kurzrock R Blood 1998 Aug 15;92(4):1150-9.

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