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中枢神经系统累及 CD4+/CD56+ 血液皮肤肿瘤:两例报告。

Central nervous system involvement in CD4+/CD56+ hematodermic neoplasm: a report of two cases.

机构信息

Department of Dermatology, Venerology and Dermatooncology, Faculty of Medicine, Semmelweis University, Mária str. 41, 1085, Budapest, Hungary.

出版信息

J Neurooncol. 2010 Apr;97(2):301-4. doi: 10.1007/s11060-009-0026-y. Epub 2009 Oct 2.

Abstract

CD4+/CD56+ hematodermic neoplasm, formerly known as blastic NK-cell lymphoma, is an uncommon, aggressive non-Hodgkin's lymphoma with cutaneous, lymph node, and bone marrow involvement at presentation. The disease is characterized by early leukemic phase; however, central nervous system involvement is rarely reported. Herein we describe two cases of CD4+/CD56+ hematodermic neoplasm with meningeal manifestation. Microscopic analysis and flow cytometry of cerebrospinal fluid proved to be diagnostic; however, imaging studies were not informative. These observations call attention to the possibility of central nervous system involvement, which could be more common than expected previously. Authors recommend routine cerebrospinal fluid analysis and prophylactic intrathecal chemotherapy in patients with this highly aggressive disease.

摘要

CD4+/CD56+ 血液皮肤肿瘤,以前称为成髓细胞性 NK 细胞淋巴瘤,是一种罕见的侵袭性非霍奇金淋巴瘤,在发病时累及皮肤、淋巴结和骨髓。该病的特征是早期白血病期;然而,中枢神经系统受累很少见。本文描述了两例表现为脑膜受累的 CD4+/CD56+ 血液皮肤肿瘤病例。脑脊液的显微镜分析和流式细胞术被证明具有诊断意义;然而,影像学研究没有提供信息。这些观察结果提醒人们注意中枢神经系统受累的可能性,这可能比之前预期的更为常见。作者建议对患有这种高度侵袭性疾病的患者进行常规脑脊液分析和预防性鞘内化疗。

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