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