Congenital Dyserythropoietic Anemia Type II: A Case Report

Abstract

Hematologic evaluation of a 29-year old man with jaundice since childhood demonstrated the characteristic findings of congenital dyserythropoietic anemia (CDA) type II. This type is characterized by ineffective erythropoiesis, multinuclearity of erythroblasts, electron-microscopic abnormalities of the red cell membrane, and abnormal serological reactions. Because of severe anemia repeated transfusions were necessary. After splenectomy thrombosis of the portal vein occurred. The patient died from hemorrhage due to ruptured esophageal varices.