ERYTHROID PROGENITORS IN CHILDHOOD PURE RED CELL APLASIA 102

Abstract

To determine the utility of marrow cultures in defining the pathophysiology and therapeutic response of pure red cell aplasia (PRCA) in children we have studied 22 patients. Patients were evaluated before specific therapies (n=20) or at the time of treatment failure (n=2). Evaluation included a medical and drug exposure history, a physical examination, marrow morphology and cytogenetics, a serological screen for autoimmune diseases, serial measurements of human parvovirus B19 specific IgM and IgG antibodies, and marrow progenitor cell cultures. Clinical follow-up was obtained to document therapeutic responses. Among 22 patients with PRCA the distribution of diseases was as follow: Transient Erythroblastopenia of Childhood (TEC) 9, Diamond-Blackfan Anemia (DBA) 4, acquired PRCA 3, PRCA combined with autoimmune hemolytic anemia 2, refractory anemia with ring sideroblasts (RARS) 2 and unclassified cases 2.