Published: 13 December 2018

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Erythropoietin and Pure Red Cell Aplasia

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Prescriber Update 39(4): 60
December 2018

Background

Pure red cell aplasia (PRCA) has been reported after treatment with recombinant human erythropoietin (r-HuEPO)¹ , particularly with Eprex² . Most cases occurred in patients with chronic kidney disease who had received Eprex subcutaneously² . Eprex is an erythropoiesis-stimulating agent used to treat or prevent anaemia of varying origins.

Pure red cell aplasia

PRCA is a rare condition of severe anaemia characterised by a very low reticulocyte count and the virtual absence of red cell precursors in the bone marrow² . All other cell lines are present and seem quantitatively and morphologically normal. Iron studies may show elevated ferritin and high transferrin saturation due to the arrested erythropoiesis and build-up of iron stores³ .

PRCA results from the induction of neutralising immunoglobulin G antibodies directed against the protein component of the recombinant erythropoietin² . These neutralising antibodies cross-react with endogenous erythropoietin, inhibiting the growth of red cell precursors in the bone marrow.

Management

If PRCA is diagnosed, r-HuEPO treatment must be discontinued immediately, and testing for erythropoietin antibodies should be considered¹ . If antibodies to erythropoietin are detected, patients should not be switched to another erythropoiesis-stimulating agent, as anti-erythropoietin antibodies cross-react with other erythropoiesis-stimulating agents¹ . Other causes of PRCA, such as viral infection (particularly parvovirus B19), malignancy, haemolytic syndromes, autoimmune disease, and seropositive arthritides may need to be excluded³ .

New Zealand reports

Up to September 2018, the Centre for Adverse Reactions Monitoring (CARM) had received 11 case reports of pure red cell aplasia after treatment with erythropoietin. The time to onset was known for 10 reports, and ranged from 1 week to 26 months (median 8.4 months).

References

1. Janssen-Cilag (New Zealand) Ltd. 2017. Eprex solution for injection New Zealand Data Sheet 24 November 2017. URL: www.medsafe.govt.nz/profs/Datasheet/e/eprexinj.pdf (accessed 9 October 2018).
2. Berns JS. 2017. Pure red cell aplasia due to anti-erythropoietin antibodies. In: UpToDate 7 August 2017. URL: www.uptodate.com/contents/pure-red-cell-aplasia-due-to-anti-erythropoietin-antibodies (accessed 9 October 2018).
3. Mohd Slim MA, Shaik R. 2013. Pure red cell aplasia associated with recombinant erythropoietin: a case report and brief review of the literature. New Zealand Medical Journal 126(1386): 106– URL: www.nzma.org.nz/journal/read-the-journal/all-issues/2010-2019/2013/vol-126-no-1386/cc-slim (accessed 30 October 2018).