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The Korean Journal of Pathology 1983;17(2): 144-152.
Sézary 증후군의 1예
Sézary Syndrome
Sézary syndrome was first described in 1983, by Sézary and Bouvrain. The classical description of this syndrome consist of generalized pruritus, exfoliative dermtitis with edema and thickening of the skin, ectropion, leonine facies, alopecia, dystrophic nails, keratoderma of the palms and soles, and lymphadenopathy associated with leukocytosis and abnormal mononuclear cells. We present a case of Sézary syndrome, in a 50-year-old male farmer with generalized exfoliative erythroderma, keratoderma of palms and soles, generalized lymphadenopathy, hepatomegaly, and pruritus, as clinical features, and severe leukocytosis (W.B.C.: 66.000㎣ of blood) with increased atypical mononuclear cells (21,120/㎣ of blood) in the peripheral blood, of which most were identified as cerebriform mononuclear cells with electromicroscopy as hematologic features. Histopathologic findings of the skin lesions revealed hyperkeratosis with parakeratosis and slight acanthosis of epidermis, moderate to marked mononuclear cells mixed with a few atypical cells in the papillary and upper dermis with epidermotropism. Axillary lymph node biopsy findings showed loss of normal architecture and marked infiltration of mononuclear cells with many atypical cells. Chemotherapy with vincristine, cyclophosphamide, adriamycin, bleomycin, and prednisolone, was done. Two weeks later, the exfoliative erythrodermic lesion and pruritus were nearly subsided and a few atypical lymphocytes (504/㎣) were observed in peripheral blood smear.
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