CHRONIC CONGENITAL AREGENERATIVE ANEMIA (PURE RED-CELL ANEMIA) ASSOCIATED WITH ISO-IMMUNIZATION BY THE BLOOD GROUP FACTOR "A"

Abstract

Chronic congenital aregenerative anemia describes a "pure red-cell" anemia in which the failure of hematopoiesis is restricted entirely to the erythrocytes without simultaneous impairment of leukocyte or platelet production. The separation of this entity from the category of the increasing number of cases designated as hypoplastic anemia will facilitate a more direct examination of the factors involved in its pathogenesis. In the case described in this paper illustrating this condition, the onset of the anemia dated to the newborn period with the clinical and hematologic features of a mild type of erythroblastosis fetalis. The mother’s blood group was O, Rh positive and that of the infant and father A, Rh positive. The anti-A serum titer in the mother reached a maximum of 1:128,000. The infant was shown to be a non-secretor. The patient, now 17 months of age, requires repeated transfusions to maintain normal blood levels. The bone marrow reveals a persistent depression of erythropoiesis but the platelet and granulocyte levels are entirely unaffected. It is postulated that prolonged depression in red blood cell production may result from an antibody directed solely against the red cells in fetal life or from the early neonatal period. This concept finds substantiation in other cases of erythroblastosis in which temporary failure of erythropoiesis as confirmed by bone marrow studies is reflected in a state of protracted anemia.