Vahancík A, Babusíková O, Ujházy P, Klobusická M, Jakubovský J, Izakovic V
1st Clinic of Internal Medicine, L. Dérer's Hospital, Bratislava, Czechoslovakia.
Neoplasma. 1988;35(1):81-9.
An 80-year-old patient has been followed for hepato- and splenomegaly, hemolytic anemia, neutropenia with lymphocytosis with large granular lymphocyte predominance in his peripheral blood, with infiltration of bone marrow, liver and probably also spleen. Determination of surface markers of proliferating lymphocytes in peripheral blood showed a mixed phenotype of T suppressor/cytotoxic and natural killer cells (SIg-, E+, T3+, T8+, EAC+, Leu7-, N901+, NK9+, VIB C5 and VIB E3-, Ia-). An in vitro cytotoxic test showed the functional inactivity of the cells tested also after human leukocyte interferon stimulation. Chromosomal analysis neither of peripheral blood lymphocytes nor of bone marrow cells proved the monoclonality marker. Following long-term prednisone therapy, the improvement of anemia, later also neutropenia accompanied by the decrease of lymphocytes has been achieved. As the disease present in our patient was distinguished only in recent years and in our country has not been reported yet, the details on its clinical, morphologic, hematologic, cytogenetic and mainly immunophenotypic characteristics are given in this paper. The problems concerning classification of the disease and determination of its biological nature are discussed.
一名80岁患者因肝脾肿大、溶血性贫血、中性粒细胞减少伴淋巴细胞增多（外周血中以大颗粒淋巴细胞为主）以及骨髓、肝脏（可能还有脾脏）浸润而接受随访。对外周血中增殖淋巴细胞的表面标志物进行测定，结果显示为T抑制/细胞毒性细胞和自然杀伤细胞的混合表型（表面免疫球蛋白阴性、E花环阳性、T3阳性、T8阳性、EAC花环阳性、Leu7阴性、N901阳性、NK9阳性、VIB C5和VIB E3阴性、Ia阴性）。体外细胞毒性试验表明，即使在人白细胞干扰素刺激后，所检测细胞的功能仍无活性。对其外周血淋巴细胞和骨髓细胞进行染色体分析，均未发现单克隆标志物。经过长期泼尼松治疗，贫血得到改善，随后中性粒细胞减少症也有所改善，同时淋巴细胞数量减少。由于我们的患者所患疾病是近年来才被区分出来的，在我国尚未见报道，因此本文给出了其临床、形态学、血液学、细胞遗传学以及主要免疫表型特征的详细信息。文中还讨论了该疾病的分类问题及其生物学性质的判定。
