Adult pure red cell aplasia at Universitas Academic Hospital, Bloemfontein, South Africa: A 9-year review
DOI:
https://doi.org/10.7196/SAMJ.2022.v112i9.16416Keywords:
HIV, Haematology, ParvovirusAbstract
Background. Pure red cell aplasia (PRCA) is characterised by severe normochromic, normocytic anaemia and partial or complete absence of reticulocytes from the peripheral blood. With bone marrow of normal cellularity, an almost complete absence of erythroblasts but preservation of other cell lines is observed. It may be congenital or acquired, with the latter presenting as a primary haematological disorder or secondary to various contributing factors. Management focuses on treatment of the underlying cause and supportive transfusions. Occasionally, immunosuppression or intravenous immunoglobulin (IVIG) is required.
Objectives. To describe the clinical characteristics, treatment and outcomes of adult patients diagnosed with PRCA at Universitas Academic Hospital (UAH) in Bloemfontein, South Africa, from 2010 to 2018.
Methods. A retrospective descriptive file review was performed. All adult patients diagnosed with PRCA and treated in the Division of
Clinical Haematology at UAH during the study period were included. Variables recorded included demographic information, clinical details of the PRCA diagnosis, classification of the PRCA, HIV and parvovirus B19 test results, results of special investigations, medical and drug history, treatment and response to treatment.
Results. Twenty-seven patients’ files were included, with a female predominance (n=22; 81.5%). The median age at diagnosis was 35 years (range 20 - 62). The median number of days from onset of symptoms to date of diagnosis was 61 days (range 27 - 114). Approximately half (n=13; 48.2%) of the patients presented with a haemoglobin concentration of 1 - 3 g/dL. Most patients (n=26; 96.3%) were infected with HIV, with 76.9% (n=20) having a suppressed viral load. Parvovirus B19 infection accounted for 44.4% of cases (n=12), and all these patients were HIV positive. Lamivudine was a probable cause of PRCA in 18.5% of cases, although the true causal relationship was uncertain. Corticosteroids and IVIG were first-line therapy in 44.4% (n=12) and 37.0% (n=10) of cases, respectively. Thirteen patients (48.2%) achieved a complete response and 7 (25.9%) a partial response, while 2 (7.4%) showed no response, with continued transfusion dependence.
Conclusion. In this population, women were disproportionately affected by PRCA. HIV was the single most important cause of acquired PRCA, which was independent of virological control. Parvovirus B19 and drugs were also important causes of acquired PRCA and played a critical part in the evaluation and work-up of PRCA. Nearly half of the patients achieved a complete response to therapy, which was sustained over 24 months.
References
Sawada K, Hirokawa M, Fujishima N. Diagnosis and management of acquired pure red cell aplasia. Hematol Oncol Clin North Am 2009;23(2):249-259. https://doi.org/10.1016/j.hoc.2009.01.009
Means RT Jr. Pure red cell aplasia. Blood 2016;128(21):51-56. https://doi.org/10.1182/ blood-2016-05-717140
Erslev AJ, Soltan A. Pure red-cell aplasia: A review. Blood Rev 1996;10(1):20-28. https://doi. org/10.1016/S0268-960X(96)90017-X
Sawada KI, Hirokawa M, Fujishima N, et al. Long-term outcome of patients with acquired primary idiopathic pure red cell aplasia receiving cyclosporine A: A nationwide cohort study in Japan for the PRCA Collaborative Study Group. Haematologica 2007;92(8):1021-1028. https://doi.org/10.3324/ haematol.11192
Sawada K, Fujishima N, Hirokawa M. Acquired pure red cell aplasia: Updated review of treatment. Br J Haematol 2008;142(4):505-514. https://doi.org/10.1111/j.1365-2141.2008.07216.x
Harris PA, Taylor R, Thielke R, Payne J, Gonzalez N, Conde JG. Research electronic data capture (REDCap) – a metadata-driven methodology and workflow process for providing translational research informatics support. J Biomed Inform 2009;42(2):377-381. https://doi.org/10.1016/j. jbi.2008.08.010
Oku K, Atsumi T, Akiyama Y, et al. Evaluation of the alternative classification criteria of systemic lupus erythematosus established by Systemic Lupus International Collaborating Clinics (SLICC). Mod Rheumatol 2018;28(4):642-648. https://doi.org/10.1080/14397595.2017.1385154
John MA, Rhemtula YA, Menezes CN, Grobusch MP. Lamivudine-induced red cell aplasia. J Med Microbiol 2008;57(8):1032-1035. https://doi.org/10.1099/jmm.0.47782-0
National Department of Health, South Africa. 2019 ART clinical guidelines for the management of HIV in adults, pregnancy, adolescents, children, infants, and neonates. https://sahivsoc.org/ Files/2019%20ART%20Guideline%2028042020%20pdf.pdf (accessed 9 November 2021).
Sanphasitvong W, Poovorawan K, Boonsuk P, Assanasen T, Nakorn TN, Poovorawan Y. Parvovirus B19 infection in HIV patient with pure red cell aplasia. Southeast Asian J Trop Med Public Health 2005;36(5):1216-1220. https://pubmed.ncbi.nlm.nih.gov/16438148/ (accessed 9 November 2021).
Intalapaporn P, Poovorawan Y, Suankratay C. Immune reconstitution syndrome associated with parvovirus B19-induced pure red cell aplasia during highly active antiretroviral therapy. J Infect 2006;53(2):e79-e82. https://doi.org/10.1016/j.jinf.2005.10.011
Morelli P, Bestetti G, Longhi E, Parravicini C, Corbellino M, Meroni L. Persistent parvovirus B19- induced anemia in an HIV-infected patient under HAART: Case report and review of literature. Eur J Clin Microbiol Infect Dis 2007;26(11):833-837. https://doi.org/10.1007/s10096-007-0360-y
Mylonakis E, Dickinson BP, Mileno MD, et al. Persistent parvovirus B19 related anemia of seven years’ duration in an HIV-infected patient: Complete remission associated with highly active antiretroviral therapy. Am J Hematol 1999;60(2):164-166. https://doi.org/10.1002/(sici)1096- 8652(199902)60:2<164::aid-ajh16>3.0.co;2-4.
Vlachaki E, Diamantidis MD, Klonizakis P, Haralambidou-Vranitsa S, Ioannidou-Papagiannaki E, Klonizakis I. Pure red cell aplasia and lymphoproliferative disorders: An infrequent association. Sci World J 2012;2012:475313. https://doi.org/10.1100/2012/475313
Corcoran C, Hardie D, Yeats J, Smuts H. Genetic variants of human parvovirus B19 in South Africa: Cocirculation of three genotypes and identification of a novel subtype of genotype 1. J Clin Microbiol 2010;48(1):137-142. https://doi.org/10.1128/JCM.00610-09
Saito Y, Sawada Y, Koga Y, et al. Isoniazid-induced pure red cell aplasia in a patient with sarcoidosis: A patient summary and review of the literature. Intern Med 2017;56(20:2753-2757. https://doi. org/10.2169/internalmedicine.8379-16
Skabelund AJ, Hauser TR, Goist KJ. Pure red cell aplasia caused by ribavirin and interferon treatment. Clin J Gastroenterol 2011;4(5):313-317. https://doi.org/10.1007/s12328-011-0235-8
Fu R, Zhang T, Liu B, et al. The clinical characteristics and therapy response of patients with acquired pure red cell aplasia. Hematology 2018;23(9):639-645. https://doi.org/10.1080/10245332.2018.1470068
Crabol Y, Terrier B, Rozenberg F, et al. 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. Clin Infect Dis 2013;56(7):968-977. https://doi.org/10.1093/cid/cis1046
Sekiguchi Y, Shimada A, Imai H, et al. A case of recurrent autoimmune hemolytic anemia during remission associated with acute pure red cell aplasia and hemophagocytic syndrome due to human parvovirus B19 infection successfully treated by steroid pulse therapy with a review of the literature. Int J Clin Exp Pathol 2014;7(5):2624-2635. https://www.semanticscholar.org/paper/A-case-of-recurrent- autoimmune-hemolytic-anemia-red-Sekiguchi-Shimada/00268166257df4b21ff8a17f7b797260d1 3e3771 (accessed 9 November 2021).
Sawada K, Hirokawa M, Fujishima N, et al. Long-term outcome of patients with acquired primary idiopathic pure red cell aplasia receiving cyclosporine A: A nationwide cohort study in Japan for the PRCA Collaborative Study Group. Haematologica 2007;92(8):1021-1028. https://doi.org/10.3324/ haematol.11192
Balasubramanian SK, Sadaps M, Thota S, et al. Rational management approach to pure red cell aplasia. Haematologica 2018;103(2):221-230. https://doi.org/10.3324/haematol.2017.175810
Sahu S, Hemlata, Verma A. Adverse events related to blood transfusion. Indian J Anaesth 2014;58(5):543-551. https://doi.org/10.4103/0019-5049.144650
Downloads
Published
Issue
Section
License
Copyright (c) 2022 S Thibile, C Barrett, S Potgieter, G Joubert, J Malherbe
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Licensing Information
The SAMJ is published under an Attribution-Non Commercial International Creative Commons Attribution (CC-BY-NC 4.0) License. Under this license, authors agree to make articles available to users, without permission or fees, for any lawful, non-commercial purpose. Users may read, copy, or re-use published content as long as the author and original place of publication are properly cited.
Exceptions to this license model is allowed for UKRI and research funded by organisations requiring that research be published open-access without embargo, under a CC-BY licence. As per the journals archiving policy, authors are permitted to self-archive the author-accepted manuscript (AAM) in a repository.
Publishing Rights
Authors grant the Publisher the exclusive right to publish, display, reproduce and/or distribute the Work in print and electronic format and in any medium known or hereafter developed, including for commercial use. The Author also agrees that the Publisher may retain in print or electronic format more than one copy of the Work for the purpose of preservation, security and back-up.
