Korean J Hematol.
2000 Nov;35(3-4):302-306.
A Case of Sezary Syndrome
- Affiliations
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- 1Department of Clinical Pathology, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea.
- 2Department of Diagnostic Pathology, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea.
- 3Department of Internal Medicine, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea.
Abstract
- Sezary syndrome (SS), one of the cutaneous T-cell lymphomas (CTCLs), is an erythrodermic variant of mycosis fungoides (MF). SS is characterized by a pruritic exfoliation or infiltrated erythroderma accompanied by circulating tumor cells with convoluted nuclei in the peripheral blood. The malignant cells in the SS usually have a mature CD4+ T-helper cell phenotype. The reported cases of SS are rare in Korea. We report a case of SS who had intractable erythroderma for 2 years and who subsequently developed generalized lymphadenopathy. The laboratory data revealed a white blood cell count of 61x109/L with 78% of lymphocytes, the majority having a convoluted or cerebriform nucleus. The skin biopsy showed Pautrier's microabscesses with dermal infiltrate of mononuclear cells containing convoluted nuclei. The lymph node biopsy demonstrated paracortical infiltration by convoluted lymphocytes. Consistent with his diagnosis, 92% of peripheral mononuclear cells expressed CD4+.
