AbstractBackground: Pure red cell aplasia (PRCA) is a rare disorder, characterized by reticulocytopenia (<1%) associated with a marked reduction of the bone marrow erythroid precursors (<5%) which is often clinically missed. Aim: To investigate the demographic profile and different causes of PRCA in the institution. Material and Methods: During a 5 year period, all cases which were reported as erythroid hyppolasia were reviewed. Based on the accepted criteria, 19 cases were included in the study. The cases were classified based on causes as primary and secondary. The clinical and laboratory profile of the cases were reviewed and followed up. Results: Among the 19 patients, two children were considered as primary PRCA as there was no other cause. The rest were secondary – 1 patient developing PRCA secondary to thymoma and 11 patients developed drug induced PRCA. 4 of them were on ART. The patient who had developed PRCA secondary to Thymoma was a 53 year old male presented with dyspnoea for more than a month. CT scan showed a mass in the anterior mediastinum which was diagnosed as thymoma and confirmed histopathologically. The four patients on ART had Zidoviduine in the regimen which is known to cause PRCA. Comments and Conclusions: The present study showed 19 patients with PRCA with diverse causes. Since PRCA is a rare disorder, haematologists should be alert to include PRCA in the differential diagnosis in cases of severe normochromic and normocytic anaemia combined with reticulocytopenia and normal production of the white blood cell and megakaryocytic lineages. The major goal in treating PRCA is to induce a remission with the recovery of erythropoiesis, thereby providing relief from transfusions and avoiding transfusion-related complications.
Keywords: Pure red cell aplasia; Thymoma; Teticulocytopenia.