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[与噬血细胞综合征相关的外周T细胞淋巴瘤:一种新近发现的实体。2例临床病理及免疫组化研究]

[Peripheral T-cell lymphoma associated with hemophagocytic syndrome: a recently identified entity. Clinico-pathologic and immunohistochemical study of 2 cases].

作者信息

Saragoni A, Falini B, Medri L, Gaudio M, Brizio R, Poggi S, Sabattini E, Pileri S

机构信息

Servizio di anatomia e istologia patologica, Ospedale Morgagni-Pierantoni di Forlì.

出版信息

Pathologica. 1990 Jul-Aug;82(1080):359-69.

PMID:2284138
Abstract

The authors report on two examples of peripheral T-cell lymphoma associated with hemophagocytic syndrome. The latter represents a recently described entity, which is characterized by pancytopenia, hepatosplenomegaly, fever and rapid downhill clinical course. Microscopic examination of different tissue samples shows bone-marrow, liver and spleen infiltration by small-medium sized lymphoid elements, which display peripheral T-cell phenotype and are associated with an exuberant hyperplasia of hemophagocytosing, benign-looking histiocytes. The clinical and pathological findings, along with the main differential diagnoses, are discussed.

摘要

作者报告了两例外周T细胞淋巴瘤合并噬血细胞综合征的病例。后者是一种最近才被描述的疾病,其特征为全血细胞减少、肝脾肿大、发热以及临床病程迅速恶化。对不同组织样本的显微镜检查显示,骨髓、肝脏和脾脏有中小等大小淋巴细胞浸润,这些细胞表现为外周T细胞表型,并伴有吞噬血细胞的良性组织细胞的旺盛增生。本文讨论了临床和病理表现以及主要的鉴别诊断。

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