Congenital Hypoplastic Anaemia Refractory to Corticosteroids but Responding to Cyclophosphamide and Antilymphocytic Globulin

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Abstract
A 7 yr old male child with congenital hypoplastic anemia (Diamond-Blackfan), having originally responded to corticosteroids, but having subsequently become refractory and erythroblastopenic since 4 yr, was treated with a combination of cyclophosphamide (CY; 2.1 g) and antilymphocytic globulin (ALG; 8 g), by the i.v. route. Erythroblastic repopulation of the bone marrow, albeit dyserythropoietic in character, reticulocytosis and erythrocytic increment took place, but only for a short period. The significance of these findings is discussed in the light of recent progress in the understanding of the disease and of its treatment.