A case of successful management with splenectomy of intractable ascites due to congenital dyserythropoietic anemia type II-induced cirrhosis

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.