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Myeloid or lymphoid neoplasm with alpha-type platelet-derived growth factor receptor gene rearrangement

MedGen UID:
415978
Concept ID:
C2827360
Neoplastic Process
Synonyms: Myeloid and Lymphoid Neoplasms with PDGFRA Rearrangement; Myeloid or lymphoid neoplasm with PDGFRA rearrangement; Myeloid-lymphoid neoplasm with PDGFRA rearrangement; Myeloid/lymphoid neoplasm with PDGFRA rearrangement; Myeloid/Lymphoid Neoplasms with PDGFRA Rearrangement
SNOMED CT: Myeloid or lymphoid neoplasm with alpha-type platelet-derived growth factor receptor gene rearrangement (450940003); Myeloid or lymphoid neoplasm with PDGFRA rearrangement (450940003); Myeloid/lymphoid neoplasm with PDGFRA rearrangement (450940003); Myeloid-lymphoid neoplasm with PDGFRA rearrangement (450940003)

Definition

Hematologic neoplasms characterized by the rearrangement of the PDGFRA gene, most often resulting in the formation of FIP1L1-PDGFRA fusion transcripts. Patients usually present with chronic eosinophilic leukemia, and less often with acute myeloid leukemia or T-lymphoblastic leukemia. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMyeloid or lymphoid neoplasm with alpha-type platelet-derived growth factor receptor gene rearrangement

Professional guidelines

PubMed

Sekeres MA, Taylor J
JAMA 2022 Sep 6;328(9):872-880. doi: 10.1001/jama.2022.14578. PMID: 36066514
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Crit Rev Oncol Hematol 2022 Mar;171:103607. Epub 2022 Jan 31 doi: 10.1016/j.critrevonc.2022.103607. PMID: 35101585
Almond LM, Charalampakis M, Ford SJ, Gourevitch D, Desai A
Clin Lymphoma Myeloma Leuk 2017 May;17(5):263-267. Epub 2017 Mar 7 doi: 10.1016/j.clml.2017.02.027. PMID: 28342811

Recent clinical studies

Etiology

Kayser S, Levis MJ
Haematologica 2023 Feb 1;108(2):308-320. doi: 10.3324/haematol.2022.280801. PMID: 36722402Free PMC Article
Shimony S, Stahl M, Stone RM
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Curr Oncol 2022 Aug 30;29(9):6245-6259. doi: 10.3390/curroncol29090491. PMID: 36135060Free PMC Article
Almond LM, Charalampakis M, Ford SJ, Gourevitch D, Desai A
Clin Lymphoma Myeloma Leuk 2017 May;17(5):263-267. Epub 2017 Mar 7 doi: 10.1016/j.clml.2017.02.027. PMID: 28342811
De Kouchkovsky I, Abdul-Hay M
Blood Cancer J 2016 Jul 1;6(7):e441. doi: 10.1038/bcj.2016.50. PMID: 27367478Free PMC Article

Diagnosis

Kayser S, Levis MJ
Haematologica 2023 Feb 1;108(2):308-320. doi: 10.3324/haematol.2022.280801. PMID: 36722402Free PMC Article
Pelcovits A, Niroula R
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Almond LM, Charalampakis M, Ford SJ, Gourevitch D, Desai A
Clin Lymphoma Myeloma Leuk 2017 May;17(5):263-267. Epub 2017 Mar 7 doi: 10.1016/j.clml.2017.02.027. PMID: 28342811

Therapy

Pollyea DA, Pratz KW, Wei AH, Pullarkat V, Jonas BA, Recher C, Babu S, Schuh AC, Dail M, Sun Y, Potluri J, Chyla B, DiNardo CD
Clin Cancer Res 2022 Dec 15;28(24):5272-5279. doi: 10.1158/1078-0432.CCR-22-1183. PMID: 36007102Free PMC Article
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Zhu AX, Abbas AR, de Galarreta MR, Guan Y, Lu S, Koeppen H, Zhang W, Hsu CH, He AR, Ryoo BY, Yau T, Kaseb AO, Burgoyne AM, Dayyani F, Spahn J, Verret W, Finn RS, Toh HC, Lujambio A, Wang Y
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J Clin Oncol 2003 Dec 15;21(24):4642-9. doi: 10.1200/JCO.2003.04.036. PMID: 14673054

Prognosis

Schneider CV, Schneider KM, Teumer A, Rudolph KL, Hartmann D, Rader DJ, Strnad P
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Leukemia 2021 Feb;35(2):440-453. Epub 2021 Jan 7 doi: 10.1038/s41375-020-01111-2. PMID: 33414482Free PMC Article
DiNardo CD, Pratz K, Pullarkat V, Jonas BA, Arellano M, Becker PS, Frankfurt O, Konopleva M, Wei AH, Kantarjian HM, Xu T, Hong WJ, Chyla B, Potluri J, Pollyea DA, Letai A
Blood 2019 Jan 3;133(1):7-17. Epub 2018 Oct 25 doi: 10.1182/blood-2018-08-868752. PMID: 30361262Free PMC Article
Rizvi S, Gores GJ
Gastroenterology 2013 Dec;145(6):1215-29. Epub 2013 Oct 15 doi: 10.1053/j.gastro.2013.10.013. PMID: 24140396Free PMC Article
Deschler B, Lübbert M
Cancer 2006 Nov 1;107(9):2099-107. doi: 10.1002/cncr.22233. PMID: 17019734

Clinical prediction guides

Fu D, Zhang B, Wu S, Zhang Y, Xie J, Ning W, Jiang H
Front Immunol 2021;12:695865. Epub 2021 May 31 doi: 10.3389/fimmu.2021.695865. PMID: 34135913Free PMC Article
Asnafi AA, Deris Zayeri Z, Shahrabi S, Zibara K, Vosughi T
J Cell Physiol 2019 May;234(5):5798-5806. Epub 2018 Nov 14 doi: 10.1002/jcp.27505. PMID: 30430567
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Recent systematic reviews

de Baat EC, Mulder RL, Armenian S, Feijen EA, Grotenhuis H, Hudson MM, Mavinkurve-Groothuis AM, Kremer LC, van Dalen EC
Cochrane Database Syst Rev 2022 Sep 27;9(9):CD014638. doi: 10.1002/14651858.CD014638.pub2. PMID: 36162822Free PMC Article
Larsson SC, Burgess S
EBioMedicine 2022 Aug;82:104154. Epub 2022 Jul 8 doi: 10.1016/j.ebiom.2022.104154. PMID: 35816897Free PMC Article
Stemler J, de Jonge N, Skoetz N, Sinkó J, Brüggemann RJ, Busca A, Ben-Ami R, Ráčil Z, Piechotta V, Lewis R, Cornely OA
Lancet Haematol 2022 May;9(5):e361-e373. doi: 10.1016/S2352-3026(22)00073-4. PMID: 35483397
Pollakova D, Andreadi A, Pacifici F, Della-Morte D, Lauro D, Tubili C
Nutrients 2021 Jun 21;13(6) doi: 10.3390/nu13062123. PMID: 34205679Free PMC Article
Smith TJ, Bohlke K, Lyman GH, Carson KR, Crawford J, Cross SJ, Goldberg JM, Khatcheressian JL, Leighl NB, Perkins CL, Somlo G, Wade JL, Wozniak AJ, Armitage JO; American Society of Clinical Oncology
J Clin Oncol 2015 Oct 1;33(28):3199-212. Epub 2015 Jul 13 doi: 10.1200/JCO.2015.62.3488. PMID: 26169616

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