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作者对8例Sézary综合征进行了临床及实验室的研究,共随访8年,其中3例因肺炎及败血症死亡。本组病例中男性6人,女性2人,年龄为47～76岁,确诊前先有皮损2～5年,曾诊断为慢性淋巴性白血病、红皮病或慢性皮炎等,均有剥脱性浸润性红皮病及淋巴结病,伴有不同程度的瘙痒。7例有掌蹠角化。部分患者有脱发,甲营养不良及面部水肿而导致眼睑外翻。无发热、体重减轻及食欲不振等全身症状。4例白细胞计数>13000/立方毫米,6例淋巴细胞绝对计数增高。治疗前周围血象中异形淋巴细胞(Sézary细胞)占2%～40%。肝功能、血生化无异常。无病变蛋白血症。肝脾扫描及胸片无特
The authors conducted a clinical and laboratory study of 8 Sézary syndromes for a total of 8 years, of which 3 died of pneumonia and sepsis. The group of 6 males and 2 females, aged 47 to 76 years old, before the diagnosis of skin lesions for 2 to 5 years, had been diagnosed as chronic lymphocytic leukemia, erythroderma or chronic dermatitis, etc., are exfoliative Invasive erythema and lymphadenopathy, accompanied by varying degrees of itching. Seven cases had palmoplantar keratosis. Some patients have hair loss, nail malnutrition and facial edema caused eyelid valgus. No fever, weight loss and loss of appetite and other systemic symptoms. 4 cases of white blood cell count> 13000 / cubic millimeter, 6 cases of lymphocyte absolute count increased. Pre-treatment peripheral blood as shaped lymphocytes (Sézary cells) accounted for 2% to 40%. Liver function, blood biochemistry no abnormalities. Disease-free leukemia. Liver and spleen scan and no special chest