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[塞扎里综合征(病例报告)]

[Sezary's syndrome (case report)].

作者信息

Miscević N, Suvajdzhić N, Jovanović V, Janković G, Radosević N, Colović M

机构信息

Institute of Haematology, University Clinical Centre, Belgrade.

出版信息

Srp Arh Celok Lek. 1995 Mar-Apr;123(3-4):102-4.

Abstract

The Sezary's syndrome is a lymphoproliferative disorder from the group of chronical lymphocytic leukaemia originated from the T-cell lineage. Authors are presenting the patient with Sezary's syndrome for the first time ever diagnosed in the Institute of Haematology. The patient, 58 years old got ill in summer 1989. with the symptoms of strong itch and erythematous papullas over the sin. In April 1990. he came to the Institute of Hematology where he was diagnosed as a Sezary's syndrome case on the evidence of generalized erythrodermia, identification of lymphocytes with cerebriform nucleus in peripheral blood and membrane-marker analysis that showed aberant post-thymic proliferation of T-lymphocytes in bone marrow. The patient wastreated with a polychemiotherapy and with "electron beam" therapy with temporary improvement, but died in January 1991 with sepsis and a hepathorenal syndrome.

摘要

蕈样肉芽肿综合征是一种源于T细胞系的慢性淋巴细胞白血病类的淋巴细胞增殖性疾病。作者首次报告了在血液学研究所诊断出的蕈样肉芽肿综合征患者。该患者为58岁男性,于1989年夏天发病,出现强烈瘙痒和皮肤红斑丘疹症状。1990年4月,他来到血液学研究所,根据全身性红皮病、外周血中出现脑回状核淋巴细胞以及膜标记分析显示骨髓中T淋巴细胞胸腺后异常增殖的证据,被诊断为蕈样肉芽肿综合征病例。该患者接受了多药化疗和“电子束”治疗,病情暂时改善,但于1991年1月死于败血症和肝肾综合征。

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