Guideline sources The following summarized guidelines for the evaluation and management of antibody-mediated pure red cell aplasia (antibody-mediated PRCA) are prepared by our editorial team based on guidelines from the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2012). 1 2 2 2 3 4 Definition Antibody-mediated PRCA is an immunological disorder seen in patients undergoing treatment with ESAs, which is characterized by the production of antibodies that inhibit the erythropoietic activity of endogenous EPO, leading to a rapidly progressive anemia. 2 Epidemiology Subcutaneous administration of recombinant EPO may lead to production of EPO-neutralizing antibodies, causing a rapid decline in blood Hgb levels and the number of circulating reticulocytes. 3 Pathophysiology In patients taking ESAs, the incidence of antibody-mediated PRCA is estimated at 14-35.8 cases per 100,000 patient-years. 2 Disease course Ther disorder is characterized by the sudden onset of severe and progressive normocytic, normochromic anemia, reticulocytopenia, and absence of erythroid precursor cells in the bone https://web.pathway.md/diseases/rec8088ZxPol53oG4 1/2 6/23/23, 2:38 AM Antibody-mediated pure red cell aplasia Pathway marrow. 2 Prognosis and risk of recurrence Treatment with pegisetanide is associated with a reduction in transfusion requirement, and freedom from transfusion in most patients after 3 months of treatment. 4 Guidelines 1. Screening and diagnosis Clinical presentation: Evaluate for possible antibody-mediated pure red-cell aplasia in patients receiving ESAs for > 8 weeks in whom: hgb concentration falls suddenly and rapidly (at a rate of 0.5 to 1.0 g/dL per week, or requirement for transfusions at a rate of approximately 1 to 2 per week) CBC shows normal platelet and white cell counts absolute reticulocyte count is < 10, 000/mL. 2. Medical management Cessation of ESA therapy: discontinue erythropoiesis-stimulated agents in patients who develop antibody-mediated PRCA. B Peginesatide therapy: administer peginesatide for the treatment of antibody-mediated PRCA. B References 1. KDIGO Executive Committee. KDIGO Clinical Practice Guideline for Anemia in Chronic Kidney Disease. KDIGO. 2012 Aug. Open 2. Macdougall IC, Casadevall N, Locatelli F et al. Incidence of erythropoietin antibody-mediated pure red cell aplasia: the Prospective Immunogenicity Surveillance Registry PRIMS . Nephrol Dial Transplant. 2015 Mar;30 3 451 60. Open 3. Casadevall N. What is antibody-mediated pure red cell aplasia PRCA ?. Nephrol Dial Transplant. 2005 May;20 Suppl 4:iv3 8. Open 4. Macdougall IC, Rossert J, Casadevall N et al. A peptide-based erythropoietin-receptor agonist for pure red-cell aplasia. N Engl J Med. 2009 Nov 5;361 19 1848 55. Open https://web.pathway.md/diseases/rec8088ZxPol53oG4 2/2