Scarisbrick J J, Child F, Spittle M, Calonje E, Russell-Jones R
Skin Tumour Unit, St John's Institute of Dermatology, St Thomas' Hospital, Lambeth Palace Rd, Westminster, London SE1 7EH, UK.
Br J Dermatol. 2000 Apr;142(4):771-5. doi: 10.1046/j.1365-2133.2000.03424.x.
We report a case of a 71-year-old male with Sézary syndrome diagnosed in 1996 who subsequently developed systemic Hodgkin's lymphoma. His only past treatment was bath psoralen plus ultraviolet A. He has since been treated with multiagent chemotherapy (ChlVPP/PABLOE) which induced a remission in his Hodgkin's disease. Eighteen months later he remains in remission from Hodgkin's disease but the Sézary syndrome remains active. He has also developed a squamous cell carcinoma on the upper lip. Sézary syndrome is a primary cutaneous T-cell lymphoma characterized by a malignant proliferation of CD4-positive cells in the skin and peripheral circulation. The CD4 count may be markedly elevated but this results from expansion of a neoplastic T-cell clone and there is a relative lymphopenia of normal T cells leading to a degree of immunoparesis. Immunosuppression is known to be associated with an increased rate of malignancies and this may account for the occurrence of Hodgkin's disease and squamous cell carcinoma in this patient with Sézary syndrome.
我们报告一例1996年诊断为Sezary综合征的71岁男性患者，该患者随后发展为系统性霍奇金淋巴瘤。他既往仅接受过补骨脂素浴加紫外线A治疗。此后，他接受了多药化疗（ChlVPP/PABLOE），化疗使他的霍奇金病得到缓解。18个月后，他的霍奇金病仍处于缓解期，但Sezary综合征仍处于活动状态。他的上唇还出现了鳞状细胞癌。Sezary综合征是一种原发性皮肤T细胞淋巴瘤，其特征是皮肤和外周循环中CD4阳性细胞的恶性增殖。CD4计数可能会显著升高，但这是由肿瘤性T细胞克隆的扩增导致的，正常T细胞相对淋巴细胞减少，导致一定程度的免疫不全。已知免疫抑制与恶性肿瘤发生率增加有关，这可能解释了该Sezary综合征患者发生霍奇金病和鳞状细胞癌的原因。
