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.
The congenital dyserythropoietic anemias comprise agroup of rare hereditary disorders of erythropoiesis,characterized by ineffective erythropoiesis as thepredominant mechanism of anemia and by characteristicmorphological aberrations of the majority of erythroblastsin the bone marrow.Congenital dyserythropoieticanemia type II is the most frequent type.All types ofcongenital dyserythropoietic anemias distinctly share ahigh incidence of iron loading.Iron accumulation occurseven in untransfused patients and can result in heartfailure and liver cirrhosis.We have reported about apatient who presented with liver cirrhosis and intractableascites caused by congenital dyserythropoietic anemiatype Ⅱ.Her clinical course was further complicatedby the development of autoimmune hemolyticanemia.Splenectomy was eventually performed whichachieved complete resolution of ascites,increase ofhemoglobin concentration and abrogation of transfusionrequirements.
