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Myeloid/lymphoid neoplasm associated with JAK2 rearrangement

MedGen UID:
1689091
•Concept ID:
C5229383
•
Neoplastic Process
Synonyms: Myeloid and Lymphoid Neoplasms with t(8;9)(p22;p24.1);PCM1-JAK2; Myeloid and/or lymphoid neoplasm with PCM1-JAK2; Myeloid and/or lymphoid neoplasm with pericentriolar material 1-janus kinase 2; Myeloid-lymphoid neoplasm with JAK2 rearrangement; myeloid/lymphoid neoplasm associated with JAK2 rearrangement; Myeloid/lymphoid neoplasm with JAK2 rearrangement; Myeloid/Lymphoid Neoplasm with JAK2-PCM1 Fusion; Myeloid/Lymphoid Neoplasm with PCM1- JAK2 Fusion; Myeloid/lymphoid neoplasm with PCM1-JAK2; Myeloid/Lymphoid Neoplasms with PCM1-JAK2; myeloid/lymphoid neoplasms with PCM1-JAK2; Myeloid/lymphoid neoplasms with PCM1-JAK2; Myeloid/Lymphoid Neoplasms with PCM1::JAK2
SNOMED CT: Myeloid and/or lymphoid neoplasm with PCM1-JAK2 (787941004); Myeloid and/or lymphoid neoplasm with pericentriolar material 1-janus kinase 2 (787941004); Myeloid and/or lymphoid neoplasm with PCM1-JAK2 (1229988003); Myeloid and/or lymphoid neoplasm with pericentriolar material 1-janus kinase 2 (1229988003); Myeloid/lymphoid neoplasm with PCM1-JAK2 (1229988003); Myeloid/lymphoid neoplasm associated with JAK2 rearrangement (1229988003); Myeloid/lymphoid neoplasm with JAK2 rearrangement (787941004); Myeloid-lymphoid neoplasm with JAK2 rearrangement (787941004)
 
Monarch Initiative: MONDO:0035121
Orphanet: ORPHA589542

Definition

Rare myeloid neoplasms characterized by eosinophilia, bone marrow findings of left-shifted erythroid predominance, lymphoid aggregates, and often myelofibrosis, and the detection of t(8;9)(p22;p24.1) and PCM1-JAK2 fusion gene. Rare cases present as T- or B-acute lymphoblastic leukemia. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMyeloid/lymphoid neoplasm associated with JAK2 rearrangement

Professional guidelines

PubMed

Clinicopathologic characteristics, genetic features, and treatment options for acute lymphoblastic leukemia with JAK2 rearrangement-A 10-case study and literature review.
Zhang L, Shah B, Zhang Y, Tashkandi H, Xiao W, Fernandez-Pol S, Vergara-Lluri M, Hussaini M, Song J, Lancet J, Moscinski L, Yun S, Lu CM, Medeiros LJ, Tang G
Hum Pathol 2023 Jun;136:1-15. Epub 2023 Mar 22 doi: 10.1016/j.humpath.2023.03.002. PMID: 36958463
World Health Organization-defined eosinophilic disorders: 2019 update on diagnosis, risk stratification, and management.
Shomali W, Gotlib J
Am J Hematol 2019 Oct;94(10):1149-1167. doi: 10.1002/ajh.25617. PMID: 31423623
World Health Organization-defined eosinophilic disorders: 2017 update on diagnosis, risk stratification, and management.
Gotlib J
Am J Hematol 2017 Nov;92(11):1243-1259. doi: 10.1002/ajh.24880. PMID: 29044676

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Myeloid/Lymphoid Neoplasms with Eosinophilia and Tyrosine Kinase Gene Fusions, 2023

Recent clinical studies

Etiology

Myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase fusion genes: A workshop report with focus on novel entities and a literature review including paediatric cases.
Saft L, Kvasnicka HM, Boudova L, Gianelli U, Lazzi S, Rozman M
Histopathology 2023 Dec;83(6):829-849. Epub 2023 Aug 8 doi: 10.1111/his.15021. PMID: 37551450
Myeloid/lymphoid neoplasms with FLT3 rearrangement.
Tang G, Tam W, Short NJ, Bose P, Wu D, Hurwitz SN, Bagg A, Rogers HJ, Hsi ED, Quesada AE, Wang W, Miranda RN, Bueso-Ramos CE, Medeiros LJ, Nardi V, Hasserjian RP, Arber DA, Orazi A, Foucar K, Wang SA
Mod Pathol 2021 Sep;34(9):1673-1685. Epub 2021 May 14 doi: 10.1038/s41379-021-00817-7. PMID: 33990705
World Health Organization-defined eosinophilic disorders: 2019 update on diagnosis, risk stratification, and management.
Shomali W, Gotlib J
Am J Hematol 2019 Oct;94(10):1149-1167. doi: 10.1002/ajh.25617. PMID: 31423623
Myeloid neoplasms with eosinophilia.
Reiter A, Gotlib J
Blood 2017 Feb 9;129(6):704-714. Epub 2016 Dec 27 doi: 10.1182/blood-2016-10-695973. PMID: 28028030
Targeting signaling pathways in acute lymphoblastic leukemia: new insights.
Harrison CJ
Hematology Am Soc Hematol Educ Program 2013;2013:118-25. doi: 10.1182/asheducation-2013.1.118. PMID: 24319172

Diagnosis

Myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase fusion genes: A workshop report with focus on novel entities and a literature review including paediatric cases.
Saft L, Kvasnicka HM, Boudova L, Gianelli U, Lazzi S, Rozman M
Histopathology 2023 Dec;83(6):829-849. Epub 2023 Aug 8 doi: 10.1111/his.15021. PMID: 37551450
Updates on eosinophilic disorders.
Tzankov A, Reichard KK, Hasserjian RP, Arber DA, Orazi A, Wang SA
Virchows Arch 2023 Jan;482(1):85-97. Epub 2022 Sep 7 doi: 10.1007/s00428-022-03402-8. PMID: 36068374
Myeloid/Lymphoid Neoplasms Associated With Eosinophilia and Rearrangements of PDGFRA, PDGFRB, or FGFR1 or With PCM1-JAK2.
Pozdnyakova O, Orazi A, Kelemen K, King R, Reichard KK, Craig FE, Quintanilla-Martinez L, Rimsza L, George TI, Horny HP, Wang SA
Am J Clin Pathol 2021 Feb 4;155(2):160-178. doi: 10.1093/ajcp/aqaa208. PMID: 33367495
World Health Organization-defined eosinophilic disorders: 2019 update on diagnosis, risk stratification, and management.
Shomali W, Gotlib J
Am J Hematol 2019 Oct;94(10):1149-1167. doi: 10.1002/ajh.25617. PMID: 31423623
Myeloid neoplasms with eosinophilia.
Reiter A, Gotlib J
Blood 2017 Feb 9;129(6):704-714. Epub 2016 Dec 27 doi: 10.1182/blood-2016-10-695973. PMID: 28028030

Therapy

World Health Organization-defined eosinophilic disorders: 2019 update on diagnosis, risk stratification, and management.
Shomali W, Gotlib J
Am J Hematol 2019 Oct;94(10):1149-1167. doi: 10.1002/ajh.25617. PMID: 31423623
Myeloid neoplasms with eosinophilia.
Reiter A, Gotlib J
Blood 2017 Feb 9;129(6):704-714. Epub 2016 Dec 27 doi: 10.1182/blood-2016-10-695973. PMID: 28028030

Prognosis

Clinicopathologic characteristics, genetic features, and treatment options for acute lymphoblastic leukemia with JAK2 rearrangement-A 10-case study and literature review.
Zhang L, Shah B, Zhang Y, Tashkandi H, Xiao W, Fernandez-Pol S, Vergara-Lluri M, Hussaini M, Song J, Lancet J, Moscinski L, Yun S, Lu CM, Medeiros LJ, Tang G
Hum Pathol 2023 Jun;136:1-15. Epub 2023 Mar 22 doi: 10.1016/j.humpath.2023.03.002. PMID: 36958463
Update on CML-Like Disorders.
Cross NCP
Clin Lymphoma Myeloma Leuk 2020 Sep;20 Suppl 1:S101-S102. doi: 10.1016/S2152-2650(20)30477-8. PMID: 32862848
World Health Organization-defined eosinophilic disorders: 2019 update on diagnosis, risk stratification, and management.
Shomali W, Gotlib J
Am J Hematol 2019 Oct;94(10):1149-1167. doi: 10.1002/ajh.25617. PMID: 31423623
Myeloid neoplasms with eosinophilia.
Reiter A, Gotlib J
Blood 2017 Feb 9;129(6):704-714. Epub 2016 Dec 27 doi: 10.1182/blood-2016-10-695973. PMID: 28028030
Targeting signaling pathways in acute lymphoblastic leukemia: new insights.
Harrison CJ
Hematology Am Soc Hematol Educ Program 2013;2013:118-25. doi: 10.1182/asheducation-2013.1.118. PMID: 24319172

Clinical prediction guides

Myeloid/lymphoid neoplasms with FLT3 rearrangement.
Tang G, Tam W, Short NJ, Bose P, Wu D, Hurwitz SN, Bagg A, Rogers HJ, Hsi ED, Quesada AE, Wang W, Miranda RN, Bueso-Ramos CE, Medeiros LJ, Nardi V, Hasserjian RP, Arber DA, Orazi A, Foucar K, Wang SA
Mod Pathol 2021 Sep;34(9):1673-1685. Epub 2021 May 14 doi: 10.1038/s41379-021-00817-7. PMID: 33990705
The diagnostic challenges and clinical course of a myeloid/lymphoid neoplasm with eosinophilia and ZBTB20-JAK2 gene fusion presenting as B-lymphoblastic leukemia.
Lee WY, Pfau RB, Choi SM, Yang J, Xiao H, Putnam EM, Ryan RJ, Bixby DL, Shao L
Cold Spring Harb Mol Case Stud 2020 Apr;6(2) Epub 2020 Apr 1 doi: 10.1101/mcs.a004937. PMID: 32238402Free PMC Article
Myeloid neoplasms with eosinophilia.
Reiter A, Gotlib J
Blood 2017 Feb 9;129(6):704-714. Epub 2016 Dec 27 doi: 10.1182/blood-2016-10-695973. PMID: 28028030

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Myeloid/Lymphoid Neoplasms with Eosinophilia and Tyrosine Kinase Gene Fusions, 2023

    Reviews

    Related information

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