mycosis fungoides and sezary syndrome. definitions mycosis fungoides: mycosis fungoides: extranodal...
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Mycosis Fungoides and Mycosis Fungoides and Sezary SyndromeSezary Syndrome
DefinitionsDefinitions
Mycosis fungoides:Mycosis fungoides:Extranodal Non-Hodgkins lymphoma of T-Extranodal Non-Hodgkins lymphoma of T-
cell origin, with primary involvement of the cell origin, with primary involvement of the skinskin
Sezary Syndrome:Sezary Syndrome:Generalized erythrodermaGeneralized erythrodermaLymphadenopathyLymphadenopathyAtypical T- cells (Sezary cells) in the Atypical T- cells (Sezary cells) in the
peripheral bloodperipheral blood
Epidemiology MFEpidemiology MF
3 cases/ 1,000,000/ year3 cases/ 1,000,000/ year<1000/year US<1000/year USPeak age 55-60Peak age 55-60Male: female 2:1Male: female 2:1
TIMycosis fungoides in the United States. Increasing incidence and descriptive epidemiology.TIMycosis fungoides in the United States. Increasing incidence and descriptive epidemiology.
AUWeinstock MA; Horm JWAUWeinstock MA; Horm JW
SOJAMA 1988 Jul 1;260(1):42-6.SOJAMA 1988 Jul 1;260(1):42-6.
ClinicalClinical
Indolent cutaneous eruption with Indolent cutaneous eruption with erythematous scaly patches or plaques, erythematous scaly patches or plaques, typically bathing trunk distributiontypically bathing trunk distribution
Heterogeneity in presentationHeterogeneity in presentation
Extracutaneous DiseaseExtracutaneous Disease
Progression to Extracutaneous disease Progression to Extracutaneous disease correlates with extent of skin diseasecorrelates with extent of skin disease
Limited patch or plaqueLimited patch or plaque very rare very rareGeneralized plaqueGeneralized plaque 8 % 8 %Tumorous or generalized Tumorous or generalized
erythrodermaerythroderma30-40%30-40%
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, 11/06
HistologyHistology
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, 11/06
Sezary CellSezary Cell
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, 11/06
TNMB classification system for TNMB classification system for mycosis fungoidesmycosis fungoides
T1 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)T2 Generalized patch/plaque (>10 percent of total skin surface) T3 TumorsT3 Tumors T4 Generalized erythrodermaT4 Generalized erythroderma N0 Lymph nodes clinically uninvolvedN0 Lymph nodes clinically uninvolved N1 Lymph nodes enlarged, histologically uninvolved (includes N1 Lymph nodes enlarged, histologically uninvolved (includes
"reactive" and "dermatopathic" nodes)"reactive" and "dermatopathic" nodes) N2 Lymph nodes clinically uninvolved but histologically involvedN2 Lymph nodes clinically uninvolved but histologically involved N3 Lymph nodes enlarged and histologically involvedN3 Lymph nodes enlarged and histologically involved M0 No visceral involvementM0 No visceral involvement M1 Visceral involvement (histologically confirmed)M1 Visceral involvement (histologically confirmed) B0 No circulating atypical (Sezary) cells (< 5 percent of B0 No circulating atypical (Sezary) cells (< 5 percent of
lymphocytes)lymphocytes) B1 Circulating atypical (Sezary) cells ( 5 percent of lymphocytes)B1 Circulating atypical (Sezary) cells ( 5 percent of lymphocytes)
Prognosis By Tumor ExtentPrognosis By Tumor Extent
Mycosis fungoides and Sézary Syndrome. Semin Oncol 1999; 26:276. figure 1, page 279.
Transformation to Large CellTransformation to Large Cell
One study of 115 pt’s; 39 % with One study of 115 pt’s; 39 % with transfomation over 12 year follow uptransfomation over 12 year follow up
Median time to transformation of 12 mMedian 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, 11/06Hoppe RT, Kim YH,Clinical features, staging, and prognosis of mycosis fungoides and Sezary syndrome, Uptodate.com, 11/06