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作者报告2例先有再生障碍性贫血(aplastic anemia,AA)的临床及形态学图象的毛细胞性白血病(hair cell leukemia,HCL)。例1:男,63岁。1976年因患流行性感冒发现全血细胞减少,血红蛋白9.3g/dl,红细胞压积28%,白细胞2.1×10~9/L,其中多形核20%,带状核5%,淋巴60%,单核12%,外周血中未见毛细胞。骨髓穿刺及活检示细胞增生减低,未见异常浸润。脾未扪及。以羟甲雄酮治疗3个月因转氨酶增高而停用。在初诊AA后约23个月血小板降至0.04×10~(12)/L,脾扫描轻度肿大。骨髓活检示细胞增生减低,脂肪间隙内有单个核细胞浸润,抗酒石酸酸性磷酸酶(TRAP)染色呈阳性,乃确诊为HCL。后患大肠杆菌败血症并中毒性休克死亡。
The authors report two cases of hair cell leukemia (HCL) that first had clinical and morphologic images of aplastic anemia (AA). Example 1: Male, 63 years old. 1976 pancytopenia was found in patients with influenza, hemoglobin 9.3g / dl, hematocrit 28%, white blood cells 2.1 × 10 ~ 9 / L, of which polymorphonuclear 20%, 5% of the nucleus, lymph 60% Mononuclear 12%, no hair cells in the peripheral blood. Bone marrow biopsy and biopsy showed reduced cell proliferation, no abnormal infiltration. Spleen not palpable. Treatment with oxymatrone 3 months due to elevated transaminases disabled. About 23 months after initial diagnosis of AA platelet decreased to 0.04 × 10 ~ (12) / L, mild splenic enlargement of the spleen. Bone marrow biopsy showed decreased cell proliferation, infiltration of mononuclear cells in the fat gap, and positive staining for tartrate-resistant acid phosphatase (TRAP), a diagnosis of HCL. After suffering from E. coli sepsis and toxic shock death.