Congenital dyserythropoietic anemia type I. Report of a case with increased erythrocyte agglutinability by anti-i serum.

Abstract

In 1967, Wendt and Heimpel 1 described a new form of macrocytic anemia in a pair of dizygotic twins. The anemia, detected in early childhood, was associated with ineffective erythropoiesis and with characteristic erythroblast morphologic features. These included multiple nuclear anomalies, megaloblastoid change, and internuclear chromatin bridges. The disease was named congenital dyserythropoietic anemia (CDA). The same authors 2 subsequently classified the disorder as CDA type I to distinguish it from a morphologically different form of congenital anemia due to ineffective erythropoiesis (CDA type II), reported independently by Crookston et al, 3 and from a third type of hereditary dyserythropoietic anemia, which had been described earlier by Wolff and von Hofe as "familial erythroid multinuclearity" 4 and by Bergstrom and Jacobsson as "hereditary benign erythroreticulosis" 5 (CDA type III). CDA type II is being observed with increasing frequency, 6-9 but only nine patients with CDA type I have been reported