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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)
 
HPO: HP:0012410
Monarch Initiative: MONDO:0001705

Definition

A type of anemia resulting from suppression of erythropoiesis with little or no abnormality of leukocyte or platelet production. Erythroblasts are virtually absent in bone marrow; however, leukocyte and platelet production show little or no reduction. [from HPO]

Conditions with this feature

Purine-nucleoside phosphorylase deficiency
MedGen UID:
75653
•Concept ID:
C0268125
•
Disease or Syndrome
Purine nucleoside phosphorylase deficiency is a rare autosomal recessive immunodeficiency disorder characterized mainly by decreased T-cell function. Some patients also have neurologic impairment (review by Aust et al., 1992).
See: Condition Record
Diamond-Blackfan anemia-like
MedGen UID:
1633207
•Concept ID:
C4693556
•
Disease or Syndrome
See: Condition Record
Bone marrow failure syndrome 5
MedGen UID:
1648380
•Concept ID:
C4748488
•
Disease or Syndrome
Bone marrow failure syndrome-5 (BMFS5) is a hematologic disorder characterized by infantile onset of severe red cell anemia requiring transfusion. Additional features include hypogammaglobulinemia, poor growth with microcephaly, developmental delay, and seizures (summary by Toki et al., 2018) For a discussion of genetic heterogeneity of BMFS, see BMFS1 (614675).
See: Condition Record
Bone marrow failure syndrome 5
Diamond-Blackfan anemia-like
Purine-nucleoside phosphorylase deficiency

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
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
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

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
Low- and intermediate-risk myelodysplastic syndrome with pure red cell aplasia.
Wang H, Niu H, Zhang T, Xing L, Shao Z, Fu R
Hematology 2021 Dec;26(1):444-446. doi: 10.1080/16078454.2021.1929694. PMID: 34153199
Pure red cell aplasia in systemic lupus erythematosus, a nationwide retrospective cohort and review of the literature.
Lobbes H, Mahévas M, Alviset S, Galicier L, Costedoat-Chalumeau N, Amoura Z, Alric L, Hot A, Durupt S, Michel M, Godeau B
Rheumatology (Oxford) 2021 Dec 24;61(1):355-366. doi: 10.1093/rheumatology/keab363. PMID: 33871586
Autoimmune cytopenias in chronic lymphocytic leukemia.
Visco C, Barcellini W, Maura F, Neri A, Cortelezzi A, Rodeghiero F
Am J Hematol 2014 Nov;89(11):1055-62. Epub 2014 Jul 7 doi: 10.1002/ajh.23785. PMID: 24912821

Diagnosis

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
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
How I manage acquired pure red cell aplasia in adults.
Gurnari C, Maciejewski JP
Blood 2021 Apr 15;137(15):2001-2009. doi: 10.1182/blood.2021010898. PMID: 33657207Free PMC Article
Unusual Anemias.
Daughety MM, DeLoughery TG
Med Clin North Am 2017 Mar;101(2):417-429. Epub 2016 Dec 10 doi: 10.1016/j.mcna.2016.09.011. PMID: 28189179
Thymic malignancies.
Loehrer PJ Sr, Wick MR
Cancer Treat Res 2001;105:277-302. doi: 10.1007/978-1-4615-1589-0_11. PMID: 11224991

Therapy

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
Atezolizumab-induced pure red cell aplasia.
Bennett R, Ruskova A
Br J Haematol 2021 Apr;193(1):10. Epub 2021 Feb 14 doi: 10.1111/bjh.17259. PMID: 33583015
Unusual Anemias.
Daughety MM, DeLoughery TG
Med Clin North Am 2017 Mar;101(2):417-429. Epub 2016 Dec 10 doi: 10.1016/j.mcna.2016.09.011. PMID: 28189179
Thymic malignancies.
Loehrer PJ Sr, Wick MR
Cancer Treat Res 2001;105:277-302. doi: 10.1007/978-1-4615-1589-0_11. PMID: 11224991
Pure red cell aplasia.
Fisch P, Handgretinger R, Schaefer HE
Br J Haematol 2000 Dec;111(4):1010-22. doi: 10.1046/j.1365-2141.2000.02429.x. PMID: 11167735

Prognosis

Somatic mutations in acquired pure red cell aplasia.
Kawakami T, Nakazawa H, Ishida F
Semin Hematol 2022 Jul;59(3):131-136. Epub 2022 Jul 16 doi: 10.1053/j.seminhematol.2022.07.001. PMID: 36115689
Prevalence of Pure Red Cell Aplasia Following Major ABO-Incompatible Hematopoietic Stem Cell Transplantation.
Zhu P, Wu Y, Cui D, Shi J, Yu J, Zhao Y, Lai X, Liu L, Xie J, Huang H, Luo Y
Front Immunol 2022;13:829670. Epub 2022 Feb 11 doi: 10.3389/fimmu.2022.829670. PMID: 35222414Free PMC Article
Low- and intermediate-risk myelodysplastic syndrome with pure red cell aplasia.
Wang H, Niu H, Zhang T, Xing L, Shao Z, Fu R
Hematology 2021 Dec;26(1):444-446. doi: 10.1080/16078454.2021.1929694. PMID: 34153199
Pure red cell aplasia in systemic lupus erythematosus, a nationwide retrospective cohort and review of the literature.
Lobbes H, Mahévas M, Alviset S, Galicier L, Costedoat-Chalumeau N, Amoura Z, Alric L, Hot A, Durupt S, Michel M, Godeau B
Rheumatology (Oxford) 2021 Dec 24;61(1):355-366. doi: 10.1093/rheumatology/keab363. PMID: 33871586
Autoimmune cytopenias in chronic lymphocytic leukemia.
Visco C, Barcellini W, Maura F, Neri A, Cortelezzi A, Rodeghiero F
Am J Hematol 2014 Nov;89(11):1055-62. Epub 2014 Jul 7 doi: 10.1002/ajh.23785. PMID: 24912821

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
Prevalence of Pure Red Cell Aplasia Following Major ABO-Incompatible Hematopoietic Stem Cell Transplantation.
Zhu P, Wu Y, Cui D, Shi J, Yu J, Zhao Y, Lai X, Liu L, Xie J, Huang H, Luo Y
Front Immunol 2022;13:829670. Epub 2022 Feb 11 doi: 10.3389/fimmu.2022.829670. PMID: 35222414Free PMC Article
Clozapine-induced anemia: A case-report
.
Eleftheriou G, Butera R, Barcella L, Falanga A
Int J Clin Pharmacol Ther 2020 May;58(5):289-292. doi: 10.5414/CP203679. PMID: 32145755
Rational management approach to pure red cell aplasia.
Balasubramanian SK, Sadaps M, Thota S, Aly M, Przychodzen BP, Hirsch CM, Visconte V, Radivoyevitch T, Maciejewski JP
Haematologica 2018 Feb;103(2):221-230. Epub 2017 Dec 7 doi: 10.3324/haematol.2017.175810. PMID: 29217782Free PMC Article

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
Is there a reason for concern or is it just hype? - A systematic literature review of the clinical consequences of switching from originator biologics to biosimilars.
Inotai A, Prins CPJ, Csanádi M, Vitezic D, Codreanu C, Kaló Z
Expert Opin Biol Ther 2017 Aug;17(8):915-926. Epub 2017 Jun 26 doi: 10.1080/14712598.2017.1341486. PMID: 28650704

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