Soler J, Bordes R, Ortũno F, Montagud M, Martorell J, Pons C, Nomdedeu J, Lopez-Lopez J J, Prat J, Rutllant M
Servei d'Hematologia, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
Br J Haematol. 1994 Mar;86(3):659-62. doi: 10.1111/j.1365-2141.1994.tb04804.x.
We report two patients with leukaemic proliferations of large granular lymphocytes. The immunophenotype study showed that the leukaemic cells were positive for CD2, CD38, CD56 and anti-HLA-DR monoclonal antibodies and negative for other T-cell (CD3, CD4, CD8) and B-cell markers (CD19, CD20 and surface immunoglobulins). The clinical course was acute and a diagnosis of aggressive natural killer cell leukaemia/lymphoma was made. No clonal rearrangements of either C beta T-cell receptor or JH immunoglobulin genes were found. Functional studies done in one patient demonstrated non-restricted cytotoxic activity after activation with IL-2. Lethal midline granuloma had been previously diagnosed in both patients. A possible relationship between this entity and the natural killer cell leukaemia is discussed.
我们报告了两名患有大颗粒淋巴细胞白血病增殖的患者。免疫表型研究表明，白血病细胞对CD2、CD38、CD56和抗HLA - DR单克隆抗体呈阳性，而对其他T细胞（CD3、CD4、CD8）和B细胞标志物（CD19、CD20和表面免疫球蛋白）呈阴性。临床病程为急性，诊断为侵袭性自然杀伤细胞白血病/淋巴瘤。未发现Cβ T细胞受体或JH免疫球蛋白基因的克隆重排。对一名患者进行的功能研究表明，用白细胞介素-2激活后具有非限制性细胞毒性活性。两名患者此前均被诊断为致死性中线肉芽肿。讨论了该实体与自然杀伤细胞白血病之间的可能关系。
