Treatment of erythropoietin-induced pure red cell aplasia: a retrospective study.
BACKGROUND
Recombinant human erythropoietin is the standard treatment for anaemia related to chronic kidney disease, and its widespread use has been favoured by a very high therapeutic index. However, since 1998, more than 200 patients worldwide with chronic kidney disease treated in this way have developed neutralising antibodies to erythropoietin, causing pure red cell aplasia. We aimed to collate clinical and pathological features in patients unequivocally shown to have erythropoietin-induced pure red cell aplasia.


METHODS
We retrospectively obtained data from the files of 47 patients with pure red cell aplasia. We assessed treatment and outcome of patients and defined recovery from pure red cell aplasia as an increase in reticulocyte counts to more than 20 000 per microL in patients who were no longer transfusion-dependent.


RESULTS
When patients developed pure red cell aplasia, all were receiving erythropoietin subcutaneously, and the product most typically prescribed was epoetin alfa (Eprex, Ortho Biotech). The median delay between start of erythropoietin treatment and occurrence of pure red cell aplasia was 11 months (IQR 7.5-14). Nine patients received no immunosuppressive treatment, and none of these recovered. Of 37 patients who received immunosuppressive therapy, 29 (78%) recovered. All six patients who received a kidney transplant recovered within 1 month, and recovery rates were between 56% and 88% in patients treated with corticosteroids, corticosteroids plus cyclophosphamide, or ciclosporin. No relapse of pure red cell aplasia happened after stopping immunosuppressive treatment, but no patient was rechallenged with erythropoietin.


CONCLUSIONS
Immunosuppressive treatment accelerates recovery from erythropoietin-induced pure red cell aplasia.
Verhelst
David
D
Department of Nephrology, Tenon Hospital, Assistance Publique-Hôpitaux de Paris, and Pierre and Marie Curie University, Paris, France.
Rossert
Jérôme
J
Casadevall
Nicole
N
Krüger
Anne
A
Eckardt
Kai-Uwe
KU
Macdougall
Iain C
IC
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000368:Aged; D000740:Anemia / Q000188:drug therapy / Q000209:etiology; D004921:Erythropoietin / Q000009:adverse effects*; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007676:Kidney Failure, Chronic / Q000150:complications; D008297:Male; D008875:Middle Aged; D011994:Recombinant Proteins; D012010:Red-Cell Aplasia, Pure / Q000139:chemically induced* / Q000628:therapy*; D012189:Retrospective Studies
D000305:Adrenal Cortex Hormones; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D011994:Recombinant Proteins; D004921:Erythropoietin