A Rising Cluster of Erythropoietin-Associated Pure Red Cell Aplasia and Subsequent Response to Roxadustat: A Case...

Patients with chronic kidney disease (CKD) develop anaemia in the setting of abnormal iron metabolism and reduced endogenous erythropoietin. Pure red cell aplasia (PRCA) is a condition that, in rare cases, has been associated with erythropoietin products, commonly prescribed in patients with CKD-related anaemia. We describe 14 cases of Epoetin-alfa associated PRCA which have resulted in significant morbidity. Our cases were predominantly male (71%) with a median age of onset 68 years old. Each presented with profound anaemia 4-48 months (median 9.5 months) after commencing subcutaneous Epoetin. Heterogeneous immunosuppression regimens were used, predominantly with either monotherapy prednisone or ciclosporin. Two patients did not receive immunosuppression. Of our 14 cases, eight have since been commenced and maintained on Roxadustat with six of these subsequently reaching target haemoglobin. Of the six not commenced on Roxadustat, only one has reached target haemoglobin. This is the first multi-patient report of Roxadustat in patients with ESA-PRCA. Our cluster of cases highlights the need for strong suspicion of acquired PRCA in patients treated with erythropoietin products who present with refractory anaemia. HIF stabilisers such as Roxadustat, which have otherwise not been licensed in New Zealand, seem to be beneficial in these cases, possibly minimising the need for heavy immunosuppression.