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伴发冯·希佩尔-林道病的T细胞淋巴瘤:一例罕见病例报告及文献复习

T-cell lymphoma with von Hippel-Lindau disease: a rare case report and review of literature.

作者信息

Lou Li-Hong, Shen Hua, Lin Jun, Yan Yi-Wen, Peng Yan, Lu Jian-Hong, Xu Qu, Jiang Hai-Qiong

机构信息

International Medical Care Center, Shanghai General Hospital, Shanghai Jiao Tong University Shanghai 200080, China.

Department of Pathology, Shanghai General Hospital, Shanghai Jiao Tong University Shanghai 200080, China.

出版信息

Int J Clin Exp Pathol. 2015 May 1;8(5):5837-43. eCollection 2015.

Abstract

von Hippel-Lindau disease (VHLD) comprises a series of complicated clinical manifestations. We hereby described a unique case of co-existing T-cell lymphoma (TCL) and confirmed VHLD. The symptoms in this 42-year-old male included fever and pancytopenia. Overall tests and examination made an infectious process unlikely. The results of bone marrow biopsy confirmed the diagnosis. The purposes we described this case were to probe into the relationship between TCL and VHLD, which was not mentioned in previously literature. Combination of clinical, radiological, immunophenotypic, pathological, and genetic data plays an important role in improving the rate of diagnosis, particularly in the challenge for diagnosis of T cell non-Hodgkin lymphoma.

摘要

冯·希佩尔-林道病(VHLD)包含一系列复杂的临床表现。我们在此描述了一例同时存在T细胞淋巴瘤(TCL)且确诊为VHLD的独特病例。该42岁男性的症状包括发热和全血细胞减少。全面的检查和检验排除了感染性疾病的可能。骨髓活检结果确诊了病情。我们描述此病例的目的是探究TCL与VHLD之间的关系,此前文献中未提及这一关系。临床、影像学、免疫表型、病理和基因数据的综合应用在提高诊断率方面发挥着重要作用,尤其是在T细胞非霍奇金淋巴瘤的诊断面临挑战时。

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