Abdul Samad K, Prasanna Moorthy K, Akhar Ali P
Department of Dermatology and Venereology Medical College, Trivandrum, Kerala, India.
Indian J Dermatol Venereol Leprol. 2002 Jul-Aug;68(4):225-6.
Sezary syndrome (SS), is described as the classical triad of pruritic erythroderma, lymphadenopathy, and presence of more than 10% of circulating Sezary cells in the peripheral blood. We report on unusual case of advanced cutaneous T - cell lymphoma with classical haematological and histopathological features of Sezary syndrome, but lacking the clinical features of erythroderma. A 66 year old man presented with asymptomatic multiple papules, plaques and nodules and with generalized lymphadenopathy. Peripheral smear showed more than 60% of Sezary cells. Skin and lymph node biopsy showed typical features of T-cell lymphoma and immunohistochemistry and CD marker studies showed the cells to be atypical T-lymphocytes. This unusual case is highlighted to denote that erythroderma need not be taken as a hard and fast criterion for diagnosing Sezary syndrome.
覃样肉芽肿（SS）被描述为具有瘙痒性红皮病、淋巴结病以及外周血中循环覃样细胞超过10%的典型三联征。我们报告了一例晚期皮肤T细胞淋巴瘤的罕见病例，该病例具有覃样肉芽肿的典型血液学和组织病理学特征，但缺乏红皮病的临床特征。一名66岁男性患者表现为无症状的多发性丘疹、斑块和结节以及全身淋巴结病。外周血涂片显示超过60%的覃样细胞。皮肤和淋巴结活检显示T细胞淋巴瘤的典型特征，免疫组织化学和CD标志物研究表明这些细胞为非典型T淋巴细胞。强调这一罕见病例是为了表明红皮病不必被视为诊断覃样肉芽肿的硬性标准。
