CD3阳性、CD4阴性、CD8阴性、TCRαβ阴性、TCRγδ阳性的颗粒淋巴细胞增殖性疾病，无淋巴细胞增多及临床症状。

CD3+, CD4-, CD8-, TCR alpha beta-, TCR gamma delta+ granular lymphocyte proliferative disorder without lymphocytosis and clinical symptoms.

作者信息

Kondo H, Uematsu M, Watanabe J, Takahashi Y, Hayashi K, Iwasaki H

机构信息

Division of Haematology and Oncology, Department of Medicine, Shimizu Kohsei Hospital, Shizuoka, Japan.

出版信息

Acta Haematol. 2000;104(1):54-6. doi: 10.1159/000041073.

DOI:10.1159/000041073

PMID:11111126

Abstract

Granular lymphocyte-proliferative disorder is characterized by a proliferation of large granular lymphocytes (LGLs). It is often associated with neutropenia, rheumatoid arthritis (RA), and pure red cell aplasia (PRCA). Phenotypic analysis has demonstrated that in most cases, the LGLs show a clonal rearrangement of the TCR alpha beta rearrangement. We are reporting a patient with TCR gamma delta LGL proliferation without clinical findings and lymphocytosis. The patient showed an expansion of the CD3+, CD16+, CD56+, and CD57+ LGL populations which involved coexpression of TCR gamma delta with TCR J gamma and J delta 1 gene rearrangement. Autoimmune manifestations, including RA and PRCA, have not appeared and the results of laboratory examinations have not changed for 1 year after the diagnosis.

摘要

颗粒淋巴细胞增殖性疾病的特征是大颗粒淋巴细胞（LGL）增殖。它常与中性粒细胞减少、类风湿性关节炎（RA）和纯红细胞再生障碍（PRCA）相关。表型分析表明，在大多数情况下，LGL显示TCRαβ重排的克隆性重排。我们报告了1例TCRγδLGL增殖患者，无临床表现和淋巴细胞增多。该患者显示CD3 +、CD16 +、CD56 +和CD57 + LGL群体扩增，涉及TCRγδ与TCR Jγ和Jδ1基因重排的共表达。包括RA和PRCA在内的自身免疫表现尚未出现，诊断后1年实验室检查结果未发生变化。