Spectrum of Pure Red Cell Aplasia in a Tertiary Care Hospital in Northeast India

Background Pure red cell aplasia (PRCA) is an uncommon disorder characterized by severe normocytic normochromic anemia, reticulocytopenia, and an absence of erythroblasts from otherwise normal bone marrow. PRCA can present as either a congenital disorder or an acquired syndrome. Methodology A total of six cases of PRCA diagnosed in a tertiary care hospital from January 2016 to December 2019 were retrospectively studied. The diagnosis of PRCA was made based on bone marrow examinations showing erythroblastopenia with essentially normal myelopoiesis and megakaryopoiesis. Results Of the six patients, one was identified as having congenital PRCA, three were classified as having primary acquired PRCA, and two were diagnosed with secondary acquired PRCA. The congenital case was an infant diagnosed with Diamond-Blackfan anemia (DBA). There were three cases of primary acquired PRCA. One was idiopathic, as no specific cause could be identified. The other primary acquired case had autoimmune hemolytic anemia (AIHA); however, no underlying cause for peripheral hemolysis or secondary causes of marrow suppression could be identified. The third primary case was myelodysplastic PRCA. The causes of secondary acquired PRCA included systemic lupus erythematosus (SLE) and thymoma. Conclusion The present study demonstrates that the causes and outcomes of PRCA are varied; however, they are similar to those observed in other similar series.