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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 (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
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
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

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
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
Pure red-cell aplasia "epidemic"--mystery completely revealed?
Locatelli F, Del Vecchio L, Pozzoni P
Perit Dial Int 2007 Jun;27 Suppl 2:S303-7. PMID: 17556324
Antibody-mediated pure red cell aplasia (PRCA): epidemiology, immunogenicity and risks.
Macdougall IC
Nephrol Dial Transplant 2005 May;20 Suppl 4:iv9-15. doi: 10.1093/ndt/gfh1087. PMID: 15827058
Aplastic anemia and pure red cell aplasia.
Dessypris EN
Curr Opin Hematol 1994 Mar;1(2):157-61. PMID: 9371275

Diagnosis

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
Acquired Pure Red Cell Aplasia.
Kr S
J Assoc Physicians India 2020 Jan;68(1):100. PMID: 31979930
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
Infection and anemia.
Kwaan HC
Infect Disord Drug Targets 2011 Feb;11(1):40-4. doi: 10.2174/187152611794407791. PMID: 21303340
Epoetin-induced pure red cell aplasia: diagnosis and treatment.
Macdougall IC
Curr Opin Nephrol Hypertens 2007 Nov;16(6):585-8. doi: 10.1097/MNH.0b013e3282f0c4bf. PMID: 18089975

Therapy

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
Pure red cell aplasia and lymphoproliferative disorders: an infrequent association.
Vlachaki E, Diamantidis MD, Klonizakis P, Haralambidou-Vranitsa S, Ioannidou-Papagiannaki E, Klonizakis I
ScientificWorldJournal 2012;2012:475313. Epub 2012 Apr 19 doi: 10.1100/2012/475313. PMID: 22593689Free PMC Article
Epoetin-induced pure red cell aplasia: diagnosis and treatment.
Macdougall IC
Curr Opin Nephrol Hypertens 2007 Nov;16(6):585-8. doi: 10.1097/MNH.0b013e3282f0c4bf. PMID: 18089975
Antibody-mediated pure red cell aplasia (PRCA): epidemiology, immunogenicity and risks.
Macdougall IC
Nephrol Dial Transplant 2005 May;20 Suppl 4:iv9-15. doi: 10.1093/ndt/gfh1087. PMID: 15827058
Antibody-mediated pure red cell aplasia (PRCA) treatment and re-treatment: multiple options.
Rossert J, Macdougall I, Casadevall N
Nephrol Dial Transplant 2005 May;20 Suppl 4:iv23-26. doi: 10.1093/ndt/gfh1090. PMID: 15827054

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
Alemtuzumab-induced red cell aplasia and other immune cytopenias: not so 'pure'.
Aitken L, Patel R, D'Rozario J, Choi P
Immunotherapy 2022 Feb;14(2):95-99. Epub 2021 Nov 8 doi: 10.2217/imt-2021-0163. PMID: 34743591
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
Antibody-mediated pure red cell aplasia (PRCA): epidemiology, immunogenicity and risks.
Macdougall IC
Nephrol Dial Transplant 2005 May;20 Suppl 4:iv9-15. doi: 10.1093/ndt/gfh1087. PMID: 15827058

Clinical prediction guides

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
Pure red-cell aplasia "epidemic"--mystery completely revealed?
Locatelli F, Del Vecchio L, Pozzoni P
Perit Dial Int 2007 Jun;27 Suppl 2:S303-7. PMID: 17556324
Antibody-mediated pure red cell aplasia (PRCA): epidemiology, immunogenicity and risks.
Macdougall IC
Nephrol Dial Transplant 2005 May;20 Suppl 4:iv9-15. doi: 10.1093/ndt/gfh1087. PMID: 15827058
Aplastic anemia and pure red cell aplasia.
Dessypris EN
Curr Opin Hematol 1994 Mar;1(2):157-61. PMID: 9371275

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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