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Pure red cell aplasia

MedGen UID:
11154
•Concept ID:
C0034902
•
Disease or Syndrome
Synonyms: Aplasia Pure Red Cell; Aplasia, Erythrocyte; Aplasia, Pure Red-Cell; Aplasias, Erythrocyte; Erythrocyte Aplasia; Erythrocyte Aplasias; Pure Red Cell Aplasia; Pure Red-Cell Aplasia; Pure Red-Cell Aplasias; Red Cell Aplasia, Pure; Red-Cell Aplasia, Pure; Red-Cell Aplasias, Pure
SNOMED CT: Red cell hypoplasia (50715003); Pure red cell aplasia (50715003); Primary red cell aplasia (50715003); Pure red cell anemia (50715003)

Definition

A disease characterized by normocytic, normochromic anemia, low hematocrit, reticulocytopenia, and selective erythroid hypoplasia. [from NCI]

Professional guidelines

PubMed

Deficiency of Adenosine Deaminase 2: Clinical Manifestations, Diagnosis, and Treatment.
Grim A, Veiga KR, Saad N
Rheum Dis Clin North Am 2023 Nov;49(4):773-787. Epub 2023 Aug 1 doi: 10.1016/j.rdc.2023.06.004. PMID: 37821195
Update on pure red cell aplasia: etiology, diagnosis, and treatment.
Means RT Jr
Clin Adv Hematol Oncol 2022 Jan;20(1):18-21. PMID: 35060958
Hepatitis A: clinical manifestations and management.
Jeong SH, Lee HS
Intervirology 2010;53(1):15-9. Epub 2010 Jan 5 doi: 10.1159/000252779. PMID: 20068336

Recent clinical studies

Etiology

Hematologic Manifestations of Parvovirus B19 Infection.
Algwaiz G, Alharbi A, Alsehaim K, Alahmari A, El Fakih R, Aljurf M
Hematol Oncol Stem Cell Ther 2023 May 23;16(4):316-322. doi: 10.56875/2589-0646.1031. PMID: 37363985
Associations Between Autoimmune Conditions and Gastric Cancer Risk Among Elderly Adults in the United States.
Song M, Camargo MC, Derkach A, Rabkin CS, Engels EA
Am J Gastroenterol 2022 Mar 1;117(3):486-490. doi: 10.14309/ajg.0000000000001622. PMID: 35029169Free PMC Article
Deficiency of Adenosine Deaminase 2 (DADA2): Updates on the Phenotype, Genetics, Pathogenesis, and Treatment.
Meyts I, Aksentijevich I
J Clin Immunol 2018 Jul;38(5):569-578. Epub 2018 Jun 27 doi: 10.1007/s10875-018-0525-8. PMID: 29951947Free PMC Article
Parvovirus B19.
Landry ML
Microbiol Spectr 2016 Jun;4(3) doi: 10.1128/microbiolspec.DMIH2-0008-2015. PMID: 27337440
Anti-erythropoietin antibodies and pure red cell aplasia.
Rossert J, Casadevall N, Eckardt KU
J Am Soc Nephrol 2004 Feb;15(2):398-406. doi: 10.1097/01.asn.0000107561.59698.42. PMID: 14747386

Diagnosis

Pure red cell aplasia: The second hundred years.
Means RT Jr
Am J Med Sci 2023 Sep;366(3):160-166. Epub 2023 Jun 14 doi: 10.1016/j.amjms.2023.06.009. PMID: 37327996
Update on pure red cell aplasia: etiology, diagnosis, and treatment.
Means RT Jr
Clin Adv Hematol Oncol 2022 Jan;20(1):18-21. PMID: 35060958
Parvovirus B19.
Landry ML
Microbiol Spectr 2016 Jun;4(3) doi: 10.1128/microbiolspec.DMIH2-0008-2015. PMID: 27337440
Hepatitis A: clinical manifestations and management.
Jeong SH, Lee HS
Intervirology 2010;53(1):15-9. Epub 2010 Jan 5 doi: 10.1159/000252779. PMID: 20068336
Human parvovirus B19.
Heegaard ED, Brown KE
Clin Microbiol Rev 2002 Jul;15(3):485-505. doi: 10.1128/CMR.15.3.485-505.2002. PMID: 12097253Free PMC Article

Therapy

Efficacy and safety of daratumumab in pure red cell aplasia after allogeneic transplantation: Dutch real-world data.
Weverling F, Roeven M, Nijssen C, Broers AEC, Dovern E, van Rhenen A, Sluis GV, Hazenberg CLE, Balen PV, Kuipers MT, de Vooght KMK, Morsink L, Kuball J, Nur E, de Witte MA
Blood Adv 2024 Apr 9;8(7):1683-1686. doi: 10.1182/bloodadvances.2023011190. PMID: 38231018Free PMC Article
Daratumumab for treatment-refractory acquired idiopathic pure red cell aplasia.
Gangat N, Bleeker J, Lynch D, Olteanu H, Letendre L, Tefferi A
Haematologica 2022 Oct 1;107(10):2523-2526. doi: 10.3324/haematol.2022.281398. PMID: 35678030Free PMC Article
Anti-erythropoietin antibodies and pure red cell aplasia.
Rossert J, Casadevall N, Eckardt KU
J Am Soc Nephrol 2004 Feb;15(2):398-406. doi: 10.1097/01.asn.0000107561.59698.42. PMID: 14747386
Azathioprine-induced pure red-cell aplasia.
Pruijt JF, Haanen JB, Hollander AA, den Ottolander GJ
Nephrol Dial Transplant 1996 Jul;11(7):1371-3. PMID: 8672045
Pure red-cell aplasia.
Krantz SB
N Engl J Med 1974 Aug 15;291(7):345-50. doi: 10.1056/NEJM197408152910707. PMID: 4604991

Prognosis

Daratumumab as a novel treatment option in refractory ITP.
Vernava I, Schmitt CA
Blood Cells Mol Dis 2023 Mar;99:102724. Epub 2023 Jan 13 doi: 10.1016/j.bcmd.2023.102724. PMID: 36669360
Clusters in Pediatric Rheumatic Diseases.
Sag E, Demir S, Ozen S
Curr Rheumatol Rep 2020 May 20;22(7):28. doi: 10.1007/s11926-020-00908-5. PMID: 32436006
Hepatitis A: clinical manifestations and management.
Jeong SH, Lee HS
Intervirology 2010;53(1):15-9. Epub 2010 Jan 5 doi: 10.1159/000252779. PMID: 20068336
Immunogenicity of biopharmaceuticals.
Kessler M, Goldsmith D, Schellekens H
Nephrol Dial Transplant 2006 Oct;21 Suppl 5:v9-12. doi: 10.1093/ndt/gfl476. PMID: 16959792
Epoetin-induced autoimmune pure red cell aplasia.
Casadevall N
Hematol J 2004;5 Suppl 3:S104-9. doi: 10.1038/sj.thj.6200433. PMID: 15190289

Clinical prediction guides

Treatment strategy for acquired pure red cell aplasia: a systematic review and meta-analysis.
Lobbes H, Lega JC, Le Guenno G, Ruivard M, Mainbourg S
Blood Adv 2023 Nov 14;7(21):6451-6465. doi: 10.1182/bloodadvances.2023010587. PMID: 37624775Free PMC Article
Hematologic Manifestations of Parvovirus B19 Infection.
Algwaiz G, Alharbi A, Alsehaim K, Alahmari A, El Fakih R, Aljurf M
Hematol Oncol Stem Cell Ther 2023 May 23;16(4):316-322. doi: 10.56875/2589-0646.1031. PMID: 37363985
Daratumumab as a novel treatment option in refractory ITP.
Vernava I, Schmitt CA
Blood Cells Mol Dis 2023 Mar;99:102724. Epub 2023 Jan 13 doi: 10.1016/j.bcmd.2023.102724. PMID: 36669360
Hepatitis A: clinical manifestations and management.
Jeong SH, Lee HS
Intervirology 2010;53(1):15-9. Epub 2010 Jan 5 doi: 10.1159/000252779. PMID: 20068336
Immunogenicity of biopharmaceuticals.
Kessler M, Goldsmith D, Schellekens H
Nephrol Dial Transplant 2006 Oct;21 Suppl 5:v9-12. doi: 10.1093/ndt/gfl476. PMID: 16959792

Recent systematic reviews

Treatment strategy for acquired pure red cell aplasia: a systematic review and meta-analysis.
Lobbes H, Lega JC, Le Guenno G, Ruivard M, Mainbourg S
Blood Adv 2023 Nov 14;7(21):6451-6465. doi: 10.1182/bloodadvances.2023010587. PMID: 37624775Free PMC Article
Hemolytic Anemia after Acute Hepatitis B Virus Infection: A Case Report and Systematic Review.
Suzuki T, Okamoto T, Kawai F, Okuyama S, Fukuda K
Intern Med 2022 Feb 15;61(4):481-488. Epub 2021 Aug 24 doi: 10.2169/internalmedicine.7690-21. PMID: 34433718Free PMC Article
Management of thymoma associated autoimmune pure red cell aplasia: Case report and systematic review of the literature.
Lesire B, Durieux V, Grigoriu B, Girard N, Berghmans T
Lung Cancer 2021 Jul;157:131-146. Epub 2021 May 11 doi: 10.1016/j.lungcan.2021.05.010. PMID: 34049719
A systematic review of paraneoplastic syndromes associated with thymoma: Treatment modalities, recurrence, and outcomes in resected cases.
Zhao J, Bhatnagar V, Ding L, Atay SM, David EA, McFadden PM, Stamnes S, Lechtholz-Zey E, Wightman SC, Detterbeck FC, Kim AW
J Thorac Cardiovasc Surg 2020 Jul;160(1):306-314.e14. Epub 2019 Dec 2 doi: 10.1016/j.jtcvs.2019.11.052. PMID: 31982129
EORTC guidelines for the use of erythropoietic proteins in anaemic patients with cancer.
Bokemeyer C, Aapro MS, Courdi A, Foubert J, Link H, Osterborg A, Repetto L, Soubeyran P
Eur J Cancer 2004 Oct;40(15):2201-16. doi: 10.1016/j.ejca.2004.07.015. PMID: 15454245

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