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在1949年,Sèzary对1939年以来的4例病人进行了仔细的研究后发表了一种新的皮肤网状细胞增生病(即“Sèzary综合征”)的详细报告。此病的临床特点为全身性红皮病伴有着色过度和表皮剥脱、狮面、掌跖鳞屑和皲裂以及甲板营养不良,并合并慢性自血病。关于Sèzary综合征在临床上是否是一种特殊的独立的疾病以及将其分类为一种真正的恶性肿瘤是否适当,资料中曾有过相当多的讨论。一些研究者认为该综合征是一种良性疾病,或者是一种非特异性的复合症状,可能并发于各种不同的淋巴增生状态,如何杰金氏病和非何杰金氏淋巴瘤。另一些研究者则认为Sèzary综合征代表蕈样肉芽肿(MF)的一种类型的临床表现,是一种恶性肿瘤。近来关于淋巴细胞膜标记的研究证实,Sèzary综合征和MF中的异常细胞均具有胸腺衍生(T细胞)的特性,支持上述解释。
In 1949, Sèzary’s careful study of four patients since 1939 published a detailed report of a new cutaneous reticulocytic (“Sèzary syndrome”). The clinical features of this disease are systemic erythroderma associated with hyperchromatin and epidermal exfoliation, lion’s surface, palmoplantar scales and chapped limbs, and deck malnutrition combined with chronic autoimmune disease. There has been a considerable amount of discussion about whether Sèzary’s syndrome is clinically a specific and independent disease and whether it is appropriate to classify it as a true malignancy. Some researchers consider the syndrome a benign condition or a nonspecific comorbid syndrome that can be complicated by a variety of different lymphoproliferative states, including how to treat patients with Parkinson’s disease and non-Hodgkin’s lymphoma. Other researchers believe that Sèzary syndrome represents a type of clinical manifestation of mycosis fungoides (MF) and is a malignant tumor. Recent studies with lymphocyte membrane markers have demonstrated that both Sèzary syndrome and abnormal cells in MF are characterized by thymus-derived (T-cells), supporting the above explanation.