Variant of congenital dyserythropoietic anemia.
Nonspherocytic hemolytic anemia was diagnosed in a 34-year-old man with jaundice since childhood. Splenectomy at the age of 8 had no influence on the anemia. Bronze diabetes was diagnosed at age 31, presumably due to hemosiderosis and secondary hemochromatosis. Iron chelation was unsuccessful in controlling iron overload, but phlebotomies proved effective without aggravating the anemia. We believe the anemia represents a variant of congenital dyserythropoietic anemia, type I.
D000328:Adult; D000742:Anemia, Dyserythropoietic, Congenital / Q000473:pathology / Q000628:therapy; D000745:Anemia, Hemolytic, Congenital / Q000473:pathology / Q000628:therapy; D001815:Bloodletting; D001853:Bone Marrow / Q000473:pathology; D006801:Humans; D008297:Male