ABSTRACT
In children, congenital pure red cell aplasia (PRCA) has been called congenital hypoplastic anemia, the Diamond-Blackfan anemia, or erythrogenesis imperfecta. Acquired primary PRCA may be acute and self-limited or may persist as a chronic refractory anemia. In children PRCA is most often acute and self-limited and is generally referred to as transient erythroblastopenia of childhood (TEC). Hematologic malignancies have been widely associated with PRCA, and with the lymphoid malignancies a high enough frequency has been reported to suggest a real relationship. A striking feature in many cases of PRCA and TEC is the presence of erythroid progenitor cells that grow normally in cell culture but do not produce new red cells in the patients. A bone marrow aspiration quickly distinguishes PRCA from a purely erythroid megaloblastic anemia or myelodysplasia with marked ineffective erythropoiesis. When extracts of thymic tumors from PRCA patients were administered to animals to induce anemia, little effect was observed.
