自然杀伤细胞白血病。1例尸检病例。

Natural killer cell leukemia. An autopsy case.

作者信息

Yatabe Y, Mori N, Hirabayashi N, Asai J

机构信息

First Department of Pathology, Nagoya University School of Medicine, Japan.

出版信息

Arch Pathol Lab Med. 1994 Dec;118(12):1201-4.

PMID:7979914

Abstract

We describe an autopsy case of natural killer cell leukemia. Leukemic cells appeared morphologically as large granular lymphocytes and expressed a restricted natural killer cell phenotype (CD2, CD38, CD56, HLA-DR). Immunogenotypic analysis revealed germline configurations of TCR-beta, TCR-gamma, and JH genes, indicating a non-T-cell or non-B-cell lineage. These features are consistent with natural killer cell leukemia, a recently proposed clinicopathologic entity. Autopsy findings were characterized by an extremely dense infiltration of leukemic cells in most organs, along with destruction of the normal architecture, corresponding to an aggressive clinical course. Hemophagocytic histiocytosis and an absence of chronic inflammatory reactions caused by recurrent infection in our case are different from morphologic features of CD3+ large granular lymphocytic leukemia. No relationship between Epstein-Barr virus and natural killer cell leukemia was observed.

摘要

我们描述了一例自然杀伤细胞白血病的尸检病例。白血病细胞在形态上表现为大颗粒淋巴细胞，并表达受限的自然杀伤细胞表型（CD2、CD38、CD56、HLA-DR）。免疫基因型分析显示TCR-β、TCR-γ和JH基因呈种系构型，表明其为非T细胞或非B细胞谱系。这些特征与自然杀伤细胞白血病相符，自然杀伤细胞白血病是最近提出的一种临床病理实体。尸检结果的特征是大多数器官中白血病细胞极度密集浸润，同时正常结构遭到破坏，这与侵袭性临床病程相对应。我们病例中的噬血细胞组织细胞增多症以及因反复感染导致的慢性炎症反应缺失与CD3 +大颗粒淋巴细胞白血病的形态学特征不同。未观察到爱泼斯坦 - 巴尔病毒与自然杀伤细胞白血病之间存在关联。