Chan W C, Check I, Schick C, Brynes R K, Kateley J, Winton E F
Blood. 1984 May;63(5):1133-40.
We report four patients with expansion of a unique population of lymphocytes that is consistently associated with neutropenia. Two patients also had rheumatoid arthritis and autoantibodies. The lymphocytes contained many cytoplasmic azurophilic granules, which possessed strong acid phosphatase activity. Multiple cytoplasmic parallel tubular arrays were observed ultrastructurally. These granular lymphocytes showed the T suppressor/cytotoxic cell phenotype (E+, OKT3+, OKT8+, OKT4-, OKM1-, OKI1-) and exhibited antibody-dependent cell-mediated cytotoxic activity but little or no natural killer cytotoxicity. They did not respond to recall antigens, concanavalin A, or pokeweed mitogen, but the cells from one patient did respond to phytohemagglutinin. No in vitro suppressor cell activity on mitogenic responses of allogeneic cells and on mixed lymphocyte cultures could be demonstrated. There was no evidence of suppression of immunoglobulin synthesis in vivo. It is uncertain that the expansion of this subset of lymphocytes represents a leukemic process. Their constant association with neutropenia, however, raises the possibility that the increase in large granular lymphocytes and neutropenia might be pathogenetically related.
我们报告了4例淋巴细胞独特群体扩增的患者，该群体始终与中性粒细胞减少相关。2例患者还患有类风湿性关节炎和自身抗体。这些淋巴细胞含有许多细胞质嗜天青颗粒，具有强酸性磷酸酶活性。超微结构观察到多个细胞质平行管状排列。这些颗粒淋巴细胞表现出T抑制/细胞毒性细胞表型（E +、OKT3 +、OKT8 +、OKT4 -、OKM1 -、OKI1 -），并表现出抗体依赖性细胞介导的细胞毒性活性，但几乎没有或没有自然杀伤细胞毒性。它们对回忆抗原、刀豆球蛋白A或商陆有丝分裂原无反应，但1例患者的细胞对植物血凝素有反应。未证明对同种异体细胞的有丝分裂反应和混合淋巴细胞培养有体外抑制细胞活性。没有体内免疫球蛋白合成受抑制的证据。尚不确定这种淋巴细胞亚群的扩增是否代表白血病过程。然而，它们与中性粒细胞减少的持续关联增加了大颗粒淋巴细胞增多与中性粒细胞减少可能在发病机制上相关的可能性。
