Prieto J, Ríos E, Parrado A, Martín A, de Blas J M, Rodríguez J M
Hospital Universitario Virgen del Rocío, Servicio Andaluz de Salud, Sevilla, Spain.
J Clin Pathol. 1996 Dec;49(12):1011-3. doi: 10.1136/jcp.49.12.1011.
The case is reported of a 45 year old woman with the rare leukaemia of natural killer cell large granular lymphocyte (NK/ LGL) type. Cytometric analysis of leukaemic blasts showed that they were positive for CD2, CD38, and CD56 antigens but negative for a series of antigens including CD3, CD7, CD16, and HLA-DR. Rearrangements of the beta T cell receptor, and heavy and kappa immunoglobulin genes were not detected and neither were chromosomal abnormalities. Leukaemic blasts developed NK cytotoxicity. The patient failed to respond to aggressive chemotherapy and died three months after diagnosis. The lack of expression of HLA-DR is an extraordinary characteristic of this case, as all cases of acute NK cell leukaemias described to date expressed HLA-DR. The immunophenotype observed in the NK cell leukaemic blasts may represent the counterpart of a hypothetical normal cell precursor in an early stage of ontogenic NK cell development.
报告了一例45岁患有罕见的自然杀伤细胞大颗粒淋巴细胞（NK/LGL）型白血病的女性病例。对白血病原始细胞的细胞计数分析显示，它们对CD2、CD38和CD56抗原呈阳性，但对包括CD3、CD7、CD16和HLA-DR在内的一系列抗原呈阴性。未检测到βT细胞受体、重链和κ免疫球蛋白基因的重排，也未发现染色体异常。白血病原始细胞产生了NK细胞毒性。患者对积极的化疗无反应，诊断后三个月死亡。HLA-DR表达缺失是该病例的一个特殊特征，因为迄今为止描述的所有急性NK细胞白血病病例均表达HLA-DR。在NK细胞白血病原始细胞中观察到的免疫表型可能代表了个体发生的NK细胞发育早期阶段一种假设的正常细胞前体的对应物。
