Spectrum of pure red cell aplasia in adult population of north-west India

Abstract

Pure red cell aplasia (PRCA) is a rare haematological disorder characterised by selective inhibition of red cell precursors in the bone marrow. We conducted a retrospective analysis of nine cases of PRCA seen in our adult haematology clinic from January 2000 to December 2003. All patients had baseline hemogram, bone marrow examination, CT scan of chest, autoimmune and infectious disease markers. The diagnosis of PRCA was made on bone marrow examination showing normal granulocytic and megakaryocytic series with erythroblastopenia. The age range was 14–68 years (median: 40 years) and a male to female ratio of 3 : 1. In five patients, the aetiology of PRCA could not be identified with available investigations and were labelled as primary PRCA. The secondary causes of PRCA included three cases of thymoma and one case of non-Hodgkin lymphoma. Out of nine patients, three patients had died and two lost to follow-up. At the last available follow-up till December 2006, three patients (one secondary and two primary PRCA) are maintaining normal haemoglobin and one patient still has active disease. The retrospective review shows that causes and outcome of PRCA in the developing world are different to those seen in the West.