Winton E F, Chan W C, Check I, Colenda K W, Bongiovanni K F, Waldmann T A
Blood. 1986 May;67(5):1427-32.
A 43-year-old male with a phenotypically homogeneous, expanded subset of T cells presented in 1981 with anemia and neutropenia. The surface antigen phenotype of 99% of the peripheral blood lymphocytes was T3+, T8+, T4-, and they were morphologically large granular lymphocytes (LGL). The same cells comprised 37% of the marrow nucleated cells. Eight months after he presented, the peripheral blood T8+, LGL diminished spontaneously, and the anemia and neutropenia completely resolved. The patient remains hematologically normal as of October 1984. To determine if the T8+, LGL represented a clonal expansion, DNA from peripheral blood lymphocytes collected and cryopreserved when the patient was neutropenic and anemic, and when he was hematologically normal, was analyzed for clonal T-cell antigen receptor gene rearrangements. Using Southern blot analysis, a clonal DNA rearrangement was demonstrated, and this clone diminished but was still demonstrable in peripheral blood lymphocytes collected in 1984. The above observations implicate the expanded T8+, LGL in the pathogenesis of the neutropenia and anemia, yet the exact mechanism remains to be elucidated.
1981年，一名43岁男性因贫血和中性粒细胞减少前来就诊，其T细胞亚群表现为表型均一且扩增。外周血淋巴细胞中99%的表面抗原表型为T3 +、T8 +、T4 -，形态上为大颗粒淋巴细胞（LGL）。同样的细胞占骨髓有核细胞的37%。就诊8个月后，外周血T8 +、LGL自发减少，贫血和中性粒细胞减少完全缓解。截至1984年10月，该患者血液学指标保持正常。为确定T8 +、LGL是否代表克隆性扩增，对患者中性粒细胞减少和贫血时以及血液学指标正常时采集并冻存的外周血淋巴细胞DNA进行分析，检测克隆性T细胞抗原受体基因重排。通过Southern印迹分析，证实存在克隆性DNA重排，该克隆在1984年采集的外周血淋巴细胞中减少但仍可检测到。上述观察结果表明扩增的T8 +、LGL与中性粒细胞减少和贫血的发病机制有关，但确切机制仍有待阐明。
