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

7. 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

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

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

10. 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)

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

12. 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):

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