Pure red cell aplasia due to anti-erythropoietin antibodies
- Jeffrey S Berns, MD
Jeffrey S Berns, MD
- Editor-in-Chief — Nephrology
- Section Editor — Dialysis
- Professor of Medicine
- Perelman School of Medicine at the University of Pennsylvania
- Section Editors
- Steve J Schwab, MD
Steve J Schwab, MD
- Editor-in-Chief — Nephrology
- Section Editor — Dialysis
- University of Tennessee Health Science Center
- Stanley L Schrier, MD
Stanley L Schrier, MD
- Editor-in-Chief — Hematology
- Section Editor — Myeloproliferative Disorders; Red Cell Disorders
- Professor of Medicine
- Stanford University School of Medicine
Acquired pure red cell aplasia (PRCA) is a rare condition of profound anemia characterized by a very low reticulocyte count and the virtual absence of erythroid precursors in the bone marrow. All other cell lines are present and seem quantitatively and morphologically normal. Many cases of acquired PRCA are idiopathic. In others, underlying conditions, such as thymoma, myelodysplastic syndromes, lymphoma, leukemia, systemic autoimmune disorders, and viral infection (ie, parvovirus B19), or treatment with drugs, such as phenytoin or chloramphenicol, are identified . (See "Acquired pure red cell aplasia in the adult".)
PRCA has also been described in patients treated with recombinant human erythropoietin (EPO), resulting from the induction of antibodies directed against the EPO molecule . Most reported cases are in patients with chronic kidney disease (CKD).
PRCA due to anti-EPO antibodies in patients with chronic renal failure will be reviewed here. A discussion of other causes of acquired PRCA is presented separately. (See "Acquired pure red cell aplasia in the adult".)
ETIOLOGY AND PATHOGENESIS
Most cases of non-erythropoietin (EPO)-related pure red cell aplasia (PRCA) are mediated by immunoglobulin G (IgG) autoantibodies or cytotoxic T lymphocytes directed against erythroid precursor or progenitor cells [1,3]. Inhibition of erythropoiesis occurs primarily between the colony forming unit–erythroid (CFU-E) and proerythroblast stages. The proerythroblast is the first morphologically identifiable erythroid precursor in normal marrow and is virtually absent in the marrow of patients with PRCA. (See "Acquired pure red cell aplasia in the adult".)
PRCA due to autoantibodies against endogenous EPO is rare in patients who have never been treated with erythropoiesis-stimulating agents (ESAs) [4-6]. The serum of patients with EPO-related PRCA inhibits the growth of erythroid progenitor cells in bone marrow cultures . Neutralizing IgG antibodies to the protein component of exogenous recombinant EPO are found that crossreact with endogenous EPO.
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- ETIOLOGY AND PATHOGENESIS
- Non-Eprex cases
- SCREENING FOR ANTI-EPO ANTIBODIES
- CLINICAL MANIFESTATIONS
- EVALUATION AND DIAGNOSIS
- Bone marrow aspirate
- Anti-EPO antibodies
- DIFFERENTIAL DIAGNOSIS
- Initial management
- Immunosuppressive therapy
- Continuous erythropoiesis receptor stimulator (CERA)
- SUMMARY AND RECOMMENDATIONS