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单纯红细胞再生障碍性贫血(简称纯红再障)是一种由多种原因引起的骨髓中幼红细胞分化增殖障碍从而导致严重贫血的疾病。本病具有以下特征:①严重的慢性正细胞正色素性或大细胞正色素性贫血,外周血网织红细胞明显减少或缺如;②骨髓中粒细胞和巨核细胞系统增殖、分化正常,但红细胞系统生成极度降低;③白细胞分类计数和血小板数正常;④无髓外造血;⑤疾病无自愈倾向。纯红再障为一少见疾病,首例由 Keznel-son 等于1922年报导。迄今,本病的病因和发病机制尚未完全清楚,治疗上也还缺少确实有效的手段。本文拟就近年来国外对此病若干问题的研究进展作一简述。
Pure red cell aplasia (referred to as pure red aplastic anemia) is a disease caused by a variety of causes of bone marrow erythroid differentiation and proliferation disorders leading to severe anemia. The disease has the following characteristics: ① severe chronic positive cell or macrophages positive pigment anemia, peripheral blood reticulocyte significantly reduced or absent; ② bone marrow cells and megakaryocytes proliferation and differentiation of normal, but the red blood cells System generated extremely reduced; ③ white blood cell count and platelet count normal; ④ non-extramedullary hematopoietic; ⑤ disease no tendency to self-healing. Pure red aopap is a rare disease, the first case reported by Keznel-son in 1922. So far, the etiology and pathogenesis of this disease have not yet been fully understood, and the treatment is still lacking in effective measures. This article is going to give a brief account of the research progress on some problems of this disease in foreign countries in recent years.