SUCCESSFUL MANAGEMENT OF CONCURRENT CONGENITAL DYSERYTHROPOIETIC ANEMIA AND AUTOIMMUNE HEMOLYTIC-ANEMIA WITH SPLENECTOMY

This first known case of concurrent congenital dyserythropoietic anaem ia (CDA) and autoimmune haemolytic anaemia (AIHA) which occurred in a hispanic male and spanned 6 years from the age of 2. Light and electro n microscopy of bone marrow erythroblasts and immunophenotyping confir med CDA; serum/eluate warm autoantibodies and positive direct antiglob ulin tests (DAT) associated with severe, episodic anaemias established AIHA. Cytogenetic analysis of bone marrow cells and peripheral blood lymphocytes ascertained sex chromosome aneuploidy (48XY, + Y, + Y). Re current, life-threatening episodes of transfusion-dependent anaemia re fractory to steroids and intravenous immune globulin, were put into st able remission at age 8 years when splenectomy successfully managed bo th disorders.