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纯白细胞再生障碍是1983年Levitt等提出的一种粒细胞生成不足所致的粒细胞减少症,其发病与补体依赖性血清抑制因子介导的粒系造血抑制或粒(?)单系祖细胞缺陷有关.目前该病报道较少,为了丰富对该病的认识,现将我们诊治的2例报告如下.1 病例报告例1:男,32岁,因头昏乏力、易感冒半年于1989年6月入院.查体除发现鼻腔呈单纯性鼻炎改变外.未见其它异常.血红蛋白126g/L,白细胞2.5×10~9/L,中性粒细胞0.54,血小板175×10~9/L.骨髓增生活跃、粒系0.23.红系0.35,淋巴系0.38,巨核细胞69个/全片.乙型肝炎血清学标志:HBsAg(+).入院后经泼尼松30mg隔日一次和补血益气中药等治疗,外周血粒细胞恢复正常.但当泼尼松逐渐减量停药后.粒细胞下降至治前水平.患者于1989年3月出院、现已随访近5年,外周血粒细胞1.2～2.1×10~9/L,血红蛋白和血小板均正常,骨髓粒系造血无明显改善,较易患上呼吸道感染.
Pure leukocyte aplasia is a neutropenia caused by Levitt et al. In 1983, which is associated with complement-dependent serum inhibitor-mediated granulocytic hematopoietic suppression or granulomatosis Defects in the current report of the disease is less, in order to enrich the understanding of the disease, now we diagnosis and treatment of 2 cases are reported as follows .1 Case Report Example 1: Male, 32 years old, due to dizziness, susceptibility to cold for six months in 1989 There were no abnormalities except hemoglobin 126g / L, leukocyte 2.5 × 10-9 / L, neutrophil 0.54, and platelet 175 × 10 ~ 9 / L in addition to finding nasal rhinitis. Myeloid hyperplasia active, grain line 0.23. Red line 0.35, Lymphoid line 0.38, Megakaryocyte 69 / Whole. Hepatitis B serological markers: HBsAg (+) .After admission by prednisone 30mg every other day and blood and Qi medicine And other treatment, peripheral blood neutrophils returned to normal.But when the dose of prednisone was gradually reduced after the reduction of neutrophils to pre-treatment level.Patients were discharged in March 1989, has been followed for nearly 5 years, peripheral blood neutrophils 1.2 ~ 2.1 × 10 ~ 9 / L, hemoglobin and platelets were normal, no significant improvement in bone marrow granulocyte hematopoiesis, more prone to breathing Infection.