Pure red cell aplasia (PRCA)

Version: 3.0
Published: 20 October 2018
Condition for which Ig use is in exceptional circumstances only

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Specific Conditions
  • Pure red cell aplasia – associated B19 infection
  • Pure red cell aplasia – autoimmune mediated
Indication for Ig Use
  • Pure red cell aplasia (PRCA) associated with Parvo B19 infection in immunocompromised patients or autoimmune mediated refractory to immunosuppressant medication
Level of Evidence Evidence of probable benefit – more research needed (Category 2a)
Description and Diagnostic Criteria Pure red cell aplasia (PRCA) is a rare syndrome of severe anaemia, reticulocytopenia and a selective deficiency of erythroid progenitors. IVIg should be considered as first-line therapy for viral PRCA associated with parvovirus B19 in immunocompromised patients. IVIg is a reasonable option for patients with immunological PRCA who have failed other therapies (e.g. prednisone or cyclosporine).
Justification for Evidence Category Whilst there is no randomised controlled trials for this condition there are many published case studies demonstrating benefit of Ig in the therapy of Parvovirus B19 associated Pure red cell aplasia (PRCA) in over 130 patients with defined Immunosuppressed states. These case series suggest doses of 2g/kg are most effective, with most response demonstrated with 1–3 doses. Several small case series suggest potential benefit in patients receiving Ig therapy for refractory PRCA following immunosuppressive therapies.
Diagnosis Requirements

A diagnosis must be made by a Haematologist.
Qualifying Criteria for Ig Therapy
  • Parvo B19 virus associated pure red cell aplasia (PRCA) in immunosuppressed patient proven by bone marrow biopsy
OR
  • Immune-mediated PRCA proven by bone marrow biopsy refractory to treatment with at least two Immunosuppressant therapies
  • OR
  • Immune-mediated PRCA proven by bone marrow biopsy and immune-suppressant medications are contraindicated or have resulted in unacceptable side effects or significant toxicity
Review Criteria for Assessing the Effectiveness of Ig Use
Review is not mandated for this indication however the following indications may be useful in assessing the effectivness of Ig therapy.
  • Recovery of bone marrow
    Reduced transfusion dependence
Dose
  • Induction Dose (IVIg) - 2 g/kg given by divided doses over 2 or 5 days.
The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

Refer to the current product information sheet for further information on dose, administration and contraindications.
 Dose Calculator

 
Bibliography
Crabol, Y, Terrier, B, Rozenberg, F, et al 2013, ‘Intravenous Immunoglobulin Therapy for Pure Red Cell Aplasia related to Human Parvovirus B19 infection: a retrospective study of 10 patients and review of the literature’, Clinical Infectious Diseases, vol. 56, no. 7, pp. 968-77. https://www.ncbi.nlm.nih.gov/pubmed/23243178

Geetha, D, Zachary, JB, Baldado, HM, et al 2000, ‘Pure red cell aplasia caused by Parvovirus B19 infection in solid organ transplant recipients: a case report and review of literature’ Clinical Transplant, vol. 14, no. 6, pp. 586-91.
https://www.ncbi.nlm.nih.gov/pubmed/11127313

Kawano, N, Nagahiro, Y, Yoshida, S, et al 2013, ‘Clinical characteristics and outcomes of 11 patients with pure red cell aplasia at a single institution over a 13-year period’, Internal Medicine, vol. 52, no. 18, pp. 2025-30. https://www.ncbi.nlm.nih.gov/pubmed/24042508

Koda, Y, Mori, T, Kato, J, et al 2013, ‘Persistent parvovirus B19 infection resulting in red cell aplasia after allogeneic hematopoietic stem cell transplantation’, Transplant Infectious Disease, vol. 15, no. 6, pp. 239-42. https://www.ncbi.nlm.nih.gov/pubmed/24134728

Koduri, PR, Kumapley, R, Valladares, J, & Teter, C 1999, ‘Chronic pure red cell aplasia caused by parvovirus B19 in AIDS: use of intravenous immunoglobulin- a report of eight patients’, American Journal of Hematology, vol. 61, no. 1, pp. 16-20. https://www.ncbi.nlm.nih.gov/pubmed/10331506

Lejeune, A, Cremer, M, Von Bernuth, et al 2014, ‘Persistent pure red cell aplasia in dizygotic twins with persistent congenital parvovirus B19 infection- remission following high dose intravenous immunoglobulin’, European Journal of Pediatrics, vol. 173, pp. 1723-1726. http://link.springer.com/article/10.1007/s00431-014-2420-5

Mouthon, L, Guillevin, L, & Tellier, Z 2005, ‘Intravenous immunoglobulins in autoimmune or parvovirus B19 mediated pure red cell aplasia’, Autoimmunity Reviews, vol. 4  no. 5, pp. 264-9. https://www.ncbi.nlm.nih.gov/pubmed/15990072

Mouthon, L, Michel, M, Gandre, C, et al 2015, ‘Costs of intravenous immunoglobulin therapy in patients with unconfirmed parvovirus B19 pure red cell aplasia’, Clinical Infectious Diseases, vol. 60, no. 3, pp.488. https://www.ncbi.nlm.nih.gov/pubmed/25336624

Nair, R, Gheith, S, & Nair, SG 2016, ‘Immunotherapy-Associated Hemolytic Anemia with Pure Red-Cell Aplasia’, New England Journal of Medicine, vol. 374, no. 11, pp. 1096-7. https://www.ncbi.nlm.nih.gov/pubmed/26981948

Ontario Regional Blood Coordinating Network 2016, ‘Ontario Intravenous Immune Globulin (IVIG) Utilization Management Guidelines’, Version 3.0. Available from: http://transfusionontario.org/en/

UK Department of Health 2011, ‘Clinical Guidelines for Immunoglobulin Use’, Second Edition Update, Available from: https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/216671/dh_131107.pdf
 
UK Department of Health 2011, ‘Clinical Guidelines for Immunoglobulin Use’, Second Edition Update, Summary Poster. Available from: http://igd.mdsas.com/wp-content/uploads/2016/04/DemandManagementPoster_v4_February2016.pdf

Zhang, M, Zhong, X, Zhang, W, et al 2015, ‘Human parvovirus B19 infection induced pure red cell aplasia in liver transplant recipients’, International Journal of Clinical Practice Supplementary, vol. 183, pp. 29-34. https://www.ncbi.nlm.nih.gov/pubmed/26177162