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首页> 外文期刊>World Journal of Gastroenterology >A case of successful management with splenectomy of intractable ascites due to congenital dyserythropoietic anemia type II-induced cirrhosis.
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A case of successful management with splenectomy of intractable ascites due to congenital dyserythropoietic anemia type II-induced cirrhosis.

机译：一例因先天性贫血性贫血引起的顽固性腹水而成功治愈顽固性腹水的病例。

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摘要

The congenital dyserythropoietic anemias comprise a group of rare hereditary disorders of erythropoiesis, characterized by ineffective erythropoiesis as the predominant mechanism of anemia and by characteristic morphological aberrations of the majority of erythroblasts in the bone marrow. Congenital dyserythropoietic anemia type II is the most frequent type. All types of congenital dyserythropoietic anemias distinctly share a high incidence of iron loading. Iron accumulation occurs even in untransfused patients and can result in heart failure and liver cirrhosis. We have reported about a patient who presented with liver cirrhosis and intractable ascites caused by congenital dyserythropoietic anemia type II. Her clinical course was further complicated by the development of autoimmune hemolytic anemia. Splenectomy was eventually performed which achieved complete resolution of ascites, increase of hemoglobin concentration and abrogation of transfusion requirements.
机译：先天性促红细胞生成性贫血包括一组罕见的红细胞生成性遗传性疾病，其特征在于无效的红细胞生成是贫血的主要机制，其特征是骨髓中大多数成红细胞的形态异常。先天性贫血性贫血II型是最常见的类型。各种类型的先天性贫血性贫血显然具有很高的铁负荷发生率。铁积累甚至发生在未输血的患者中，并可能导致心力衰竭和肝硬化。我们已经报道了一位患者，该患者因II型先天性促红细胞生成性贫血而出现肝硬化和顽固性腹水。自身免疫性溶血性贫血的发展使她的临床过程更加复杂。最终进行了脾切除术，从而完全消除了腹水，增加了血红蛋白浓度并取消了输血要求。

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