McDaniel H L, MacPherson B R, Tindle B H, Lunde J H
Department of Pathology, University of Vermont College of Medicine, Burlington.
Arch Pathol Lab Med. 1992 Mar;116(3):242-8.
Lymphoproliferative disorders of granular lymphocytes (LDGLs) represent a family of diseases that are morphologically similar but diverse with regard to immunophenotype, function, and clonality. In this article, we report three informative cases and propose a modification of the current classification of LDGLs. Our first case is an example of natural killer cell LDGLs (CD2+, CD3-, CD16+, CD57+/-). Based on a review of the literature, we suggest that natural killer cell LDGLs can be divided into two subgroups (types 1 and 2) according to the expression of CD57. Reduced expression of CD57 may distinguish between patients with a poorer prognosis. The remaining two cases illustrate examples of T-cell LDGLs (CD2+, CD3+, CD8+, CD57+) that differ mainly in their expression of CD16. The CD16+ T-cell LDGLs (type 1) usually show a clonal rearrangement of the T-cell receptor-beta chain gene, whereas CD16- T-cell LDGLs (type 2) may show a germline configuration, suggesting a reactive rather than a neoplastic process. Pathologists should differentiate LDGLs from other chronic lymphoproliferative diseases, since most cases evolve slowly and aggressive cytoreductive therapy is usually unwarranted.
颗粒淋巴细胞的淋巴增殖性疾病（LDGLs）是一类在形态上相似，但在免疫表型、功能和克隆性方面存在差异的疾病。在本文中，我们报告了三例具有参考价值的病例，并提议对当前LDGLs的分类进行修订。我们的首例病例是自然杀伤细胞LDGLs（CD2 +、CD3 -、CD16 +、CD57 + / -）的一个实例。基于对文献的回顾，我们建议自然杀伤细胞LDGLs可根据CD57的表达分为两个亚组（1型和2型）。CD57表达降低可能预示患者预后较差。其余两例说明了T细胞LDGLs（CD2 +、CD3 +、CD8 +、CD57 +）的实例，它们主要在CD16的表达上有所不同。CD16 + T细胞LDGLs（1型）通常显示T细胞受体β链基因的克隆重排，而CD16 - T细胞LDGLs（2型）可能显示种系构型，提示这是一个反应性而非肿瘤性过程。病理学家应将LDGLs与其他慢性淋巴细胞增殖性疾病区分开来，因为大多数病例进展缓慢，通常无需进行积极的细胞减灭治疗。
