Pure red cell aplasia in systemic lupus erythematosus, a nationwide retrospective cohort and review of the literature

Abstract

Objectives To characterize the clinical and biological course, management and response to treatment in Systemic Lupus Erythematosus (SLE)-associated Pure Red Cell Aplasia (PRCA). Methods Nationwide multicentre retrospective cohort study. From 2006 to 2018, we included adults with a diagnosis of PRCA supported by bone-marrow examination and SLE or biologic manifestations of SLE after ruling out parvovirus B19 infection. Results We enrolled 24 patients (20 women). SLE was diagnosed before PRCA for 14 patients (median delay 81 months). At PRCA diagnosis, mean age, haemoglobin level and reticulocyte and differential erythroblast count were 39.2 ± 13.2 years, 62 ± 20 g/L, 9.1 ± 7.6 x 109/L and 2.8 ± 2.5%, respectively. Eleven (45%) patients experienced multiple PRCA flares (median 6, range: 2-11). Corticosteroid therapy resulted in only three complete sustained responses, and 19 (79%) patients required immunosuppressive agents with highly variable regimens. After a median follow-up of 76 months (range 13-173), 17 (71%) patients showed complete response for PRCA, five (21%) partial response and two (8%) treatment failure. In total, 21 (87%) patients required red-blood-cell transfusion; five had a diagnosis of transfusion-related iron overload. Eighteen (75%) patients experienced severe infectious events requiring hospitalization. Conclusion SLE-associated PRCA is a severe condition. Repeated red-blood-cell transfusions and several lines of immunosuppressant therapy are mostly required with high risk of severe infectious events and iron overload. Despite sustained response for PRCA and SLE obtained in most patients, the best therapeutic strategy remains to be determined.

A 46-Year-Old Thai Woman with Secondary Acquired Pure Red Cell Aplasia Due to Treatment with Recombinant Erythropoietin While on Dialysis for End-Stage Renal Disease Who Recovered Following ABO-Incompatible Kidney Transplantation

“…In this case, our suspicions were based on her underlying autoimmune disease and past exposure to 2 types of rHuEPO (continuous erythropoietin receptor activator [CERA] and biosimilar erythropoietin alpha). PRCA is rare in patients with SLE [ 16 , 17 ]. A retrospective study [ 17 ] we performed January 2010 to December 2017 in our center showed that nearly 50% of the etiology was ESA-related PRCA.…”

“…The pathogenesis of PRCA in SLE is hypothesized to be related to autoantibody formation targeting erythroblasts, erythroidprogenitors, and erythropoietin [ 18 , 19 ]. From a recent retrospective cohort study [ 16 ], the treatment response rates to immunosuppressive drugs were 75%, 100%, 50%, 75%, 60% in azathioprine, cyclophosphamide, cyclosporin, mycophenolic acid, and rituximab, respectively. The reciprocating increase in Hb varied depending on the immunosuppressive regimen.…”