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皮肤T细胞淋巴瘤

Cutaneous T-cell lymphoma.

作者信息

Schein P S, Macdonald J S, Edelson R

出版信息

Cancer. 1976 Oct;38(4):1859-61. doi: 10.1002/1097-0142(197610)38:4<1859::aid-cncr2820380466>3.0.co;2-s.

Abstract

A comparison of the detailed features and natural history of 12 patients with "Sézary Syndrome" with those in patients with classical "mycosis fungoides" has demonstrated that, with the exception of a leukemic phase in the former, the two syndromes are indistinguishable. Both disorders result from the proliferation of malignant thymus-derived lymphocytes with progressive involvement of skin, lymph nodes, and visceral organs; actual transition between these two syndromes have been documented. "Sézary Syndrome" and "mycosis fungoides" appear to represent variant clinical expressions of the same disease process. It is proposed that the present confusing and archaic terminology should be discarded and that the designation "cutaneous T-cell lymphoma, with or without leukemic phase" should be used in all future descriptions of this disorder.

摘要

对12例“塞扎里综合征”患者与经典“蕈样肉芽肿”患者的详细特征及自然病史进行比较后发现,除前者存在白血病期外,这两种综合征难以区分。这两种疾病均由源自胸腺的恶性淋巴细胞增殖引起,皮肤、淋巴结和内脏器官会逐渐受累;已有文献记载这两种综合征之间会实际发生转变。“塞扎里综合征”和“蕈样肉芽肿”似乎代表了同一疾病过程的不同临床表型。有人提议,应摒弃目前令人困惑且陈旧的术语,在今后对该疾病的所有描述中均使用“伴有或不伴有白血病期的皮肤T细胞淋巴瘤”这一名称。

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