Mycosis Fungoides and Sezary Syndrome1 1Definitionsl lMycosis fungoides:l lExtranodal Non-Hodgkins lymphoma of T-cell origin, with primary involvement of the skinl lSezary Syndrome:l lGeneralized erythrodermal lLymphadenopathyl lAtypical T- cells (Sezary cells) in the peripheral blood2 2Epidemiology MFl l3 cases/ 1,000,000/ year1000/year USl lPeak age 55-60l lMale: female 2:1TIMycosis fungoides in the United States. Increasing incidence and descriptive epidemiology.TIMycosis fungoides in the United States. Increasing incidence and descriptive epidemiology. AUWeinstock MA; Horm JW AUWeinstock MA; Horm JW SOJAMA 1988 Jul 1;260(1):42-6. SOJAMA 1988 Jul 1;260(1):42-6.3 3Clinicall lIndolent cutaneous eruption with erythematous scaly patches or plaques, typically bathing trunk distributionl lHeterogeneity in presentation4 45 56 6Extracutaneous Diseasel lProgression to Extracutaneous disease correlates with extent of skin diseasel lLimited patch or plaque very rarel lGeneralized plaque 8 %l lTumorous or generalized erythroderma30-40%Hoppe RT, Kim YH,Clinical features, staging, and prognosis of mycosis fungoides and Sezary syndrome, Uptodate.com, 11/06Hoppe RT, Kim YH,Clinical features, staging, and prognosis of mycosis fungoides and Sezary syndrome, Uptodate.com, 11/067 7HistologyHoppe RT, Kim YH,Clinical features, staging, and prognosis of mycosis fungoides and Sezary syndrome, Uptodate.com, 11/06Hoppe RT, Kim YH,Clinical features, staging, and prognosis of mycosis fungoides and Sezary syndrome, Uptodate.com, 11/068 8Sezary CellHoppe RT, Kim YH,Clinical features, staging, and prognosis of mycosis fungoides and Sezary syndrome, Uptodate.com, 11/06Hoppe RT, Kim YH,Clinical features, staging, and prognosis of mycosis fungoides and Sezary syndrome, Uptodate.com, 11/069 9TNMB classification system for mycosis fungoidesl lT1 Limited patch/plaque ( 10 percent of total skin surface)T1 Limited patch/plaque (10 percent of total skin surface)T2 Generalized patch/plaque (10 percent of total skin surface)l lT3 TumorsT3 Tumorsl lT4 Generalized erythrodermaT4 Generalized erythrodermal lN0 Lymph nodes clinically uninvolvedN0 Lymph nodes clinically uninvolvedl lN1 Lymph nodes enlarged, histologically uninvolved (includes N1 Lymph nodes enlarged, histologically uninvolved (includes reactive and dermatopathic nodes)reactive and dermatopathic nodes)l lN2 Lymph nodes clinically uninvolved but histologically involvedN2 Lymph nodes clinically uninvolved but histologically involvedl lN3 Lymph nodes enlarged and histologically involvedN3 Lymph nodes enlarged and histologically involvedl lM0 No visceral involvementM0 No visceral involvementl lM1 Visceral involvement (histologically confirmed)M1 Visceral involvement (histologically confirmed)l lB0 No circulating atypical (Sezary) cells ( 5 percent of B0 No circulating atypical (Sezary) cells ( 5 percent of lymphocytes)lymphocytes)l lB1 Circulating atypical (Sezary) cells ( 5 percent of lymphocytes)B1 Circulating atypical (Sezary) cells ( 5 percent of lymphocytes)1010Prognosis By Tumor ExtentMycosis fungoides and Szary Syndrome. Semin Oncol 1999; 26:276. figure 1, page 279. 1111Transformation to Large Celll lOne study of 115 pts; 39 % with One study of 115 pts; 39 % with transfomation over 12 year follow uptransfomation over 12 year follow upl lMedian time to transformation of 12 mMedian time to transformation of 12 m1212Hoppe RT, Kim YH,Clinical features, staging, and prognosis of mycosis fungoides and Sezary syndrome, Uptodate.com, 11/06Hoppe RT, Kim YH,Clinical features, staging, and prognosis of mycosis fungoides and Sezary syndrome, Uptodate.com, 11/061313