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Refractory cytopenia of childhood

MedGen UID:
417430
Concept ID:
C2826323
Neoplastic Process
Synonyms: RCC; Refractory Cytopenia of Childhood; refractory cytopenia of childhood
 
Monarch Initiative: MONDO:0044874

Definition

A myeloid disorder that occurs in childhood and is characterized by persistent cytopenia, dysplastic changes in at least two hematopoietic lineages or in 10% or more of cells in one hematopoietic lineage, less than 5% blasts in bone marrow, and less than 2% blasts in peripheral blood, absence of bone marrow fibrosis, and absence of history of prior cytotoxic chemotherapy or radiation therapy. In a subset of cases, somatic mutations or cytogenetic abnormalities can be identified. Other cases are the result of pre-existing disorders with germline mutations, including Fanconi anemia, Shwachman-Diamond syndrome, and dyskeratosis congenita. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRefractory cytopenia of childhood

Professional guidelines

PubMed

Yoshida N
Int J Hematol 2024 Mar;119(3):240-247. Epub 2023 Mar 3 doi: 10.1007/s12185-023-03564-4. PMID: 36867357
Hasegawa D, Chen X, Hirabayashi S, Ishida Y, Watanabe S, Zaike Y, Tsuchida M, Masunaga A, Yoshimi A, Hama A, Kojima S, Ito M, Nakahata T, Manabe A
Br J Haematol 2014 Sep;166(5):758-66. Epub 2014 Jun 4 doi: 10.1111/bjh.12955. PMID: 24894311
Baumann I, Führer M, Behrendt S, Campr V, Csomor J, Furlan I, de Haas V, Kerndrup G, Leguit RJ, De Paepe P, Noellke P, Niemeyer C, Schwarz S
Histopathology 2012 Jul;61(1):10-7. Epub 2012 Mar 28 doi: 10.1111/j.1365-2559.2011.04156.x. PMID: 22458667

Recent clinical studies

Etiology

Rudelius M, Weinberg OK, Niemeyer CM, Shimamura A, Calvo KR
Virchows Arch 2023 Jan;482(1):113-130. Epub 2022 Nov 29 doi: 10.1007/s00428-022-03447-9. PMID: 36445482
Iwafuchi H
J Clin Exp Hematop 2018;58(2):68-86. doi: 10.3960/jslrt.18018. PMID: 29998978Free PMC Article
Locatelli F, Strahm B
Blood 2018 Mar 29;131(13):1406-1414. Epub 2018 Feb 8 doi: 10.1182/blood-2017-09-765214. PMID: 29438960
Hasle H
Hematology Am Soc Hematol Educ Program 2016 Dec 2;2016(1):598-604. doi: 10.1182/asheducation-2016.1.598. PMID: 27913534Free PMC Article
Chatterjee T, Choudhry VP
Indian J Pediatr 2013 Sep;80(9):764-71. Epub 2013 Aug 3 doi: 10.1007/s12098-013-1130-8. PMID: 23912822

Diagnosis

Yoshida N
Int J Hematol 2024 Mar;119(3):240-247. Epub 2023 Mar 3 doi: 10.1007/s12185-023-03564-4. PMID: 36867357
Chisholm KM, Bohling SD
Clin Lab Med 2023 Dec;43(4):639-655. Epub 2023 Aug 7 doi: 10.1016/j.cll.2023.06.005. PMID: 37865508
Rudelius M, Weinberg OK, Niemeyer CM, Shimamura A, Calvo KR
Virchows Arch 2023 Jan;482(1):113-130. Epub 2022 Nov 29 doi: 10.1007/s00428-022-03447-9. PMID: 36445482
Galaverna F, Ruggeri A, Locatelli F
Curr Opin Oncol 2018 Nov;30(6):402-408. doi: 10.1097/CCO.0000000000000488. PMID: 30222640
Locatelli F, Strahm B
Blood 2018 Mar 29;131(13):1406-1414. Epub 2018 Feb 8 doi: 10.1182/blood-2017-09-765214. PMID: 29438960

Therapy

Ito M, Yagasaki H, Kanezawa K, Shimozawa K, Hirai M, Morioka I
Sci Rep 2021 Jul 12;11(1):14263. doi: 10.1038/s41598-021-93646-2. PMID: 34253777Free PMC Article
Hasegawa D
Int J Hematol 2016 Apr;103(4):360-4. Epub 2016 Mar 3 doi: 10.1007/s12185-016-1965-7. PMID: 26939774
Wu J, Cheng Y, Zhang L
Leuk Res 2015 Dec;39(12):1347-52. Epub 2015 Sep 21 doi: 10.1016/j.leukres.2015.09.012. PMID: 26427728
Forester CM, Sartain SE, Guo D, Harris MH, Weinberg OK, Fleming MD, London WB, Williams DA, Hofmann I
Am J Hematol 2015 Apr;90(4):320-6. Epub 2015 Mar 2 doi: 10.1002/ajh.23937. PMID: 25580823Free PMC Article
Aalbers AM, van den Heuvel-Eibrink MM, Baumann I, Dworzak M, Hasle H, Locatelli F, De Moerloose B, Schmugge M, Mejstrikova E, Nováková M, Zecca M, Zwaan CM, Te Marvelde JG, Langerak AW, van Dongen JJ, Pieters R, Niemeyer CM, van der Velden VH
Haematologica 2015 Mar;100(3):315-23. Epub 2014 Nov 25 doi: 10.3324/haematol.2014.107706. PMID: 25425683Free PMC Article

Prognosis

Ito M, Yagasaki H, Kanezawa K, Shimozawa K, Hirai M, Morioka I
Sci Rep 2021 Jul 12;11(1):14263. doi: 10.1038/s41598-021-93646-2. PMID: 34253777Free PMC Article
Hasegawa D
Int J Hematol 2016 Apr;103(4):360-4. Epub 2016 Mar 3 doi: 10.1007/s12185-016-1965-7. PMID: 26939774
Forester CM, Sartain SE, Guo D, Harris MH, Weinberg OK, Fleming MD, London WB, Williams DA, Hofmann I
Am J Hematol 2015 Apr;90(4):320-6. Epub 2015 Mar 2 doi: 10.1002/ajh.23937. PMID: 25580823Free PMC Article
Chatterjee T, Choudhry VP
Indian J Pediatr 2013 Sep;80(9):764-71. Epub 2013 Aug 3 doi: 10.1007/s12098-013-1130-8. PMID: 23912822
Yin CC, Medeiros LJ, Bueso-Ramos CE
Int J Lab Hematol 2010 Oct;32(5):461-76. Epub 2010 Jul 7 doi: 10.1111/j.1751-553X.2010.01246.x. PMID: 20626469Free PMC Article

Clinical prediction guides

Marchesi RF, Velloso EDRP, Garanito MP, Leal AM, Siqueira SAC, Azevedo Neto RS, Rocha V, Zerbini MCN
Ann Diagn Pathol 2020 Apr;45:151459. Epub 2019 Dec 30 doi: 10.1016/j.anndiagpath.2019.151459. PMID: 32000075
Locatelli F, Strahm B
Blood 2018 Mar 29;131(13):1406-1414. Epub 2018 Feb 8 doi: 10.1182/blood-2017-09-765214. PMID: 29438960
Hasegawa D
Int J Hematol 2016 Apr;103(4):360-4. Epub 2016 Mar 3 doi: 10.1007/s12185-016-1965-7. PMID: 26939774
Forester CM, Sartain SE, Guo D, Harris MH, Weinberg OK, Fleming MD, London WB, Williams DA, Hofmann I
Am J Hematol 2015 Apr;90(4):320-6. Epub 2015 Mar 2 doi: 10.1002/ajh.23937. PMID: 25580823Free PMC Article
Chatterjee T, Choudhry VP
Indian J Pediatr 2013 Sep;80(9):764-71. Epub 2013 Aug 3 doi: 10.1007/s12098-013-1130-8. PMID: 23912822

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