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Chronic Myelomonocytic Leukemia with Eosinophilia

MedGen UID:
232364
•Concept ID:
C1333045
•
Neoplastic Process
Synonym: CMML with Eosinophilia

Definition

A chronic myelomonocytic leukemia characterized by a peripheral blood eosinophil count of equal or greater than 1.5x10E9/L, and absence of PDGFRA or PDGFRB gene abnormalities. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChronic Myelomonocytic Leukemia with Eosinophilia

Professional guidelines

PubMed

Atypical chronic myeloid leukemia and myelodysplastic/myeloproliferative neoplasm, not otherwise specified: 2023 update on diagnosis, risk stratification, and management.
Patnaik MM, Tefferi A
Am J Hematol 2023 Apr;98(4):681-689. Epub 2023 Jan 4 doi: 10.1002/ajh.26828. PMID: 36601682
World health organization classification, evaluation, and genetics of the myeloproliferative neoplasm variants.
Vardiman J, Hyjek E
Hematology Am Soc Hematol Educ Program 2011;2011:250-6. doi: 10.1182/asheducation-2011.1.250. PMID: 22160042
Atypical myeloproliferative disorders: diagnosis and management.
Tefferi A, Elliott MA, Pardanani A
Mayo Clin Proc 2006 Apr;81(4):553-63. doi: 10.4065/81.4.553. PMID: 16610578

Recent clinical studies

Etiology

Atypical chronic myeloid leukemia and myelodysplastic/myeloproliferative neoplasm, not otherwise specified: 2023 update on diagnosis, risk stratification, and management.
Patnaik MM, Tefferi A
Am J Hematol 2023 Apr;98(4):681-689. Epub 2023 Jan 4 doi: 10.1002/ajh.26828. PMID: 36601682
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
Systemic mastocytosis in the elderly.
Butterfield JH, Weiler CR
Am J Hematol 2013 May;88(5):406-8. Epub 2013 Mar 27 doi: 10.1002/ajh.23426. PMID: 23436294
World health organization classification, evaluation, and genetics of the myeloproliferative neoplasm variants.
Vardiman J, Hyjek E
Hematology Am Soc Hematol Educ Program 2011;2011:250-6. doi: 10.1182/asheducation-2011.1.250. PMID: 22160042
Molecular classification and pathogenesis of eosinophilic disorders: 2005 update.
Gotlib J
Acta Haematol 2005;114(1):7-25. doi: 10.1159/000085559. PMID: 15995322

Diagnosis

Atypical chronic myeloid leukemia and myelodysplastic/myeloproliferative neoplasm, not otherwise specified: 2023 update on diagnosis, risk stratification, and management.
Patnaik MM, Tefferi A
Am J Hematol 2023 Apr;98(4):681-689. Epub 2023 Jan 4 doi: 10.1002/ajh.26828. PMID: 36601682
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
Chronic myelomonocytic leukemia with ETV6-ABL1 rearrangement and SMC1A mutation.
Cessna MH, Paulraj P, Hilton B, Sadre-Bazzaz K, Szankasi P, Cluff A, Patel JL, Hoda D, Toydemir RM
Cancer Genet 2019 Oct;238:31-36. Epub 2019 Jul 13 doi: 10.1016/j.cancergen.2019.07.004. PMID: 31425923
Eosinophilia in Hematologic Disorders.
Falchi L, Verstovsek S
Immunol Allergy Clin North Am 2015 Aug;35(3):439-52. doi: 10.1016/j.iac.2015.04.004. PMID: 26209894Free PMC Article
Hypereosinophilic syndrome and clonal eosinophilia: point-of-care diagnostic algorithm and treatment update.
Tefferi A, Gotlib J, Pardanani A
Mayo Clin Proc 2010 Feb;85(2):158-64. Epub 2010 Jan 6 doi: 10.4065/mcp.2009.0503. PMID: 20053713Free PMC Article

Therapy

Eosinophilia in Hematologic Disorders.
Falchi L, Verstovsek S
Immunol Allergy Clin North Am 2015 Aug;35(3):439-52. doi: 10.1016/j.iac.2015.04.004. PMID: 26209894Free PMC Article
Patients with myeloid malignancies bearing PDGFRB fusion genes achieve durable long-term remissions with imatinib.
Cheah CY, Burbury K, Apperley JF, Huguet F, Pitini V, Gardembas M, Ross DM, Forrest D, Genet P, Rousselot P, Patton N, Smith G, Dunbar CE, Ito S, Aguiar RC, Odenike O, Gimelfarb A, Cross NC, Seymour JF
Blood 2014 Jun 5;123(23):3574-7. Epub 2014 Mar 31 doi: 10.1182/blood-2014-02-555607. PMID: 24687085Free PMC Article
Decitabine in myelodysplastic syndromes and chronic myelomonocytic leukemia: Argentinian/South Korean multi-institutional clinical experience.
Iastrebner M, Jang JH, Nucifora E, Kim K, Sackmann F, Kim DH, Orlando S, Jung CW, Basquiera A, Klein G, Santini F, Bernard HI, Korin J, Taborda G
Leuk Lymphoma 2010 Dec;51(12):2250-7. Epub 2010 Oct 7 doi: 10.3109/10428194.2010.524324. PMID: 20929328
Hypereosinophilic syndrome and clonal eosinophilia: point-of-care diagnostic algorithm and treatment update.
Tefferi A, Gotlib J, Pardanani A
Mayo Clin Proc 2010 Feb;85(2):158-64. Epub 2010 Jan 6 doi: 10.4065/mcp.2009.0503. PMID: 20053713Free PMC Article
Imatinib therapy in clonal eosinophilic disorders, including systemic mastocytosis.
Tefferi A, Pardanani A
Int J Hematol 2004 Jun;79(5):441-7. doi: 10.1532/ijh97.04046. PMID: 15239393

Prognosis

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
Chronic myelomonocytic leukemia with ETV6-ABL1 rearrangement and SMC1A mutation.
Cessna MH, Paulraj P, Hilton B, Sadre-Bazzaz K, Szankasi P, Cluff A, Patel JL, Hoda D, Toydemir RM
Cancer Genet 2019 Oct;238:31-36. Epub 2019 Jul 13 doi: 10.1016/j.cancergen.2019.07.004. PMID: 31425923
Novel recurrent mutations in ethanolamine kinase 1 (ETNK1) gene in systemic mastocytosis with eosinophilia and chronic myelomonocytic leukemia.
Lasho TL, Finke CM, Zblewski D, Patnaik M, Ketterling RP, Chen D, Hanson CA, Tefferi A, Pardanani A
Blood Cancer J 2015 Jan 23;5(1):e275. doi: 10.1038/bcj.2014.94. PMID: 25615281Free PMC Article
Systemic mastocytosis in the elderly.
Butterfield JH, Weiler CR
Am J Hematol 2013 May;88(5):406-8. Epub 2013 Mar 27 doi: 10.1002/ajh.23426. PMID: 23436294
The relationship between the myelodysplastic syndromes and the myeloproliferative disorders.
Bain BJ
Leuk Lymphoma 1999 Aug;34(5-6):443-9. doi: 10.3109/10428199909058471. PMID: 10492067

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
Two myeloid leukemia cases with rare FLT3 fusions.
Zhang H, Paliga A, Hobbs E, Moore S, Olson S, Long N, Dao KT, Tyner JW
Cold Spring Harb Mol Case Stud 2018 Dec;4(6) Epub 2018 Dec 17 doi: 10.1101/mcs.a003079. PMID: 30559310Free PMC Article
Novel recurrent mutations in ethanolamine kinase 1 (ETNK1) gene in systemic mastocytosis with eosinophilia and chronic myelomonocytic leukemia.
Lasho TL, Finke CM, Zblewski D, Patnaik M, Ketterling RP, Chen D, Hanson CA, Tefferi A, Pardanani A
Blood Cancer J 2015 Jan 23;5(1):e275. doi: 10.1038/bcj.2014.94. PMID: 25615281Free PMC Article
Systemic mastocytosis in the elderly.
Butterfield JH, Weiler CR
Am J Hematol 2013 May;88(5):406-8. Epub 2013 Mar 27 doi: 10.1002/ajh.23426. PMID: 23436294
ETV6 gene rearrangements in hematopoietic malignant disorders.
Wlodarska I, Mecucci C, Baens M, Marynen P, van den Berghe H
Leuk Lymphoma 1996 Oct;23(3-4):287-95. doi: 10.3109/10428199609054831. PMID: 9031109

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