Pure red cell aplasia followed by disseminated intravascular coagulation in a haemodialysis patient receiving erythropoietin-β

Pure red cell aplasia (PRCA) is a rare disorder that leads to severe anaemia with selective erythroblastopenia. PRCA may be secondary to drugs or infections with viruses such as parvovirus B19, which is known to cause the depletion of red blood cell precursors; in other cases, it is associated with thymoma, B – or T –cell lymphoproliferative disorders and other malignancies. In addition, PRCA is sometimes associated with autoimmune diseases (e.g. systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) [ 1 ]. Although PRCA is very rare, the number of reported cases has dramatically increased in recent years, predominantly in patients with chronic kidney disease (CKD)–associated anaemia receiving subcutaneous injections of recombinant epoetin (rEpo) [ 1 , 2 ]. In this setting, PRCA is clearly associated with anti-erythropoietin antibodies (AEA), which have frequently been detected in the sera of affected patients and have been shown to be involved in the pathogenesis of the disease. AEA have also been studied in patients with PRCA associated with autoimmune diseases, such as SLE, RA and Sjögren's syndrome; some studies have shown that they test positive in a significant proportion of SLE patients [ 3 ]; however, these results have not been confirmed by other authors [ 4 ]. Therefore, the prevalence of AEA in autoimmune conditions is still unclear and their potential pathogenetic role needs to be elucidated. We describe here a case of PRCA associated with rEpo-β administration in a haemodialysed patient with a history of RA, who also manifested a clinical picture of disseminated intravascular coagulation (DIC).