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Chronic myelomonocytic leukemia(CMML)

MedGen UID:
44125
•Concept ID:
C0023480
•
Neoplastic Process
Synonym: CMML
SNOMED CT: Chronic myelomonocytic leukemia (128831004); Chronic myelomonocytic leukemia (127225006)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
HPO: HP:0012325
Monarch Initiative: MONDO:0020311
OMIM®: 607785
Orphanet: ORPHA98823

Definition

A myelodysplastic/myeloproliferative neoplasm which is characterized by persistent monocytosis, absence of a Philadelphia chromosome and BCR/ABL fusion gene, fewer than 20 percent blasts in the bone marrow and blood, myelodysplasia, and absence of PDGFRA or PDGFRB rearrangement. [from HPO]

Conditions with this feature

14q32 duplication syndrome
MedGen UID:
896239
•Concept ID:
C4225449
•
Disease or Syndrome
A rare chromosomal anomaly syndrome resulting from the partial duplication of the long arm of chromosome 14 that results in a predisposition to a number of adult-onset myeloproliferative neoplasms, including acute myeloid leukaemia, chronic myelomonocytic leukaemia and myeloproliferative neoplasms especially essential thrombocythemia. Progression to myelofibrosis and secondary acute myeloid leukaemia can be observed.
See: Condition Record
14q32 duplication syndrome

Professional guidelines

PubMed

Chronic myelomonocytic leukemia: 2024 update on diagnosis, risk stratification and management.
Patnaik MM, Tefferi A
Am J Hematol 2024 Jun;99(6):1142-1165. Epub 2024 Mar 7 doi: 10.1002/ajh.27271. PMID: 38450850Free PMC Article
Chronic myelomonocytic leukemia: 2022 update on diagnosis, risk stratification, and management.
Patnaik MM, Tefferi A
Am J Hematol 2022 Mar 1;97(3):352-372. Epub 2022 Jan 27 doi: 10.1002/ajh.26455. PMID: 34985762
Proposed diagnostic criteria for classical chronic myelomonocytic leukemia (CMML), CMML variants and pre-CMML conditions.
Valent P, Orazi A, Savona MR, Patnaik MM, Onida F, van de Loosdrecht AA, Haase D, Haferlach T, Elena C, Pleyer L, Kern W, Pemovska T, Vladimer GI, Schanz J, Keller A, Lübbert M, Lion T, Sotlar K, Reiter A, De Witte T, Pfeilstöcker M, Geissler K, Padron E, Deininger M, Orfao A, Horny HP, Greenberg PL, Arber DA, Malcovati L, Bennett JM
Haematologica 2019 Oct;104(10):1935-1949. Epub 2019 May 2 doi: 10.3324/haematol.2019.222059. PMID: 31048353Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Myelodysplastic Syndromes, 2024

Recent clinical studies

Etiology

Chronic myelomonocytic leukemia: 2024 update on diagnosis, risk stratification and management.
Patnaik MM, Tefferi A
Am J Hematol 2024 Jun;99(6):1142-1165. Epub 2024 Mar 7 doi: 10.1002/ajh.27271. PMID: 38450850Free PMC Article
Advances in myelodysplastic/myeloproliferative neoplasms.
Prakash S, Arber DA, Bueso-Ramos C, Hasserjian RP, Orazi A
Virchows Arch 2023 Jan;482(1):69-83. Epub 2022 Dec 5 doi: 10.1007/s00428-022-03465-7. PMID: 36469102
How I diagnose and treat chronic myelomonocytic leukemia.
Patnaik MM
Haematologica 2022 Jul 1;107(7):1503-1517. doi: 10.3324/haematol.2021.279500. PMID: 35236051Free PMC Article
Chronic myelomonocytic leukemia: 2022 update on diagnosis, risk stratification, and management.
Patnaik MM, Tefferi A
Am J Hematol 2022 Mar 1;97(3):352-372. Epub 2022 Jan 27 doi: 10.1002/ajh.26455. PMID: 34985762
Clinical and biological implications of driver mutations in myelodysplastic syndromes.
Papaemmanuil E, Gerstung M, Malcovati L, Tauro S, Gundem G, Van Loo P, Yoon CJ, Ellis P, Wedge DC, Pellagatti A, Shlien A, Groves MJ, Forbes SA, Raine K, Hinton J, Mudie LJ, McLaren S, Hardy C, Latimer C, Della Porta MG, O'Meara S, Ambaglio I, Galli A, Butler AP, Walldin G, Teague JW, Quek L, Sternberg A, Gambacorti-Passerini C, Cross NC, Green AR, Boultwood J, Vyas P, Hellstrom-Lindberg E, Bowen D, Cazzola M, Stratton MR, Campbell PJ; Chronic Myeloid Disorders Working Group of the International Cancer Genome Consortium
Blood 2013 Nov 21;122(22):3616-27; quiz 3699. Epub 2013 Sep 12 doi: 10.1182/blood-2013-08-518886. PMID: 24030381Free PMC Article

Diagnosis

Chronic myelomonocytic leukemia: 2024 update on diagnosis, risk stratification and management.
Patnaik MM, Tefferi A
Am J Hematol 2024 Jun;99(6):1142-1165. Epub 2024 Mar 7 doi: 10.1002/ajh.27271. PMID: 38450850Free PMC Article
Advances in myelodysplastic/myeloproliferative neoplasms.
Prakash S, Arber DA, Bueso-Ramos C, Hasserjian RP, Orazi A
Virchows Arch 2023 Jan;482(1):69-83. Epub 2022 Dec 5 doi: 10.1007/s00428-022-03465-7. PMID: 36469102
How I diagnose and treat chronic myelomonocytic leukemia.
Patnaik MM
Haematologica 2022 Jul 1;107(7):1503-1517. doi: 10.3324/haematol.2021.279500. PMID: 35236051Free PMC Article
Chronic myelomonocytic leukemia: 2022 update on diagnosis, risk stratification, and management.
Patnaik MM, Tefferi A
Am J Hematol 2022 Mar 1;97(3):352-372. Epub 2022 Jan 27 doi: 10.1002/ajh.26455. PMID: 34985762
Leukemia Cutis.
Barry D, Schmieder A
N Engl J Med 2021 Sep 30;385(14):1316. doi: 10.1056/NEJMicm2103771. PMID: 34587388

Therapy

Current status and research directions in acute myeloid leukemia.
Kantarjian H, Borthakur G, Daver N, DiNardo CD, Issa G, Jabbour E, Kadia T, Sasaki K, Short NJ, Yilmaz M, Ravandi F
Blood Cancer J 2024 Sep 19;14(1):163. doi: 10.1038/s41408-024-01143-2. PMID: 39300079Free PMC Article
Characteristics and clinical outcomes of patients with myeloid malignancies and DDX41 variants.
Bataller A, Loghavi S, Gerstein Y, Bazinet A, Sasaki K, Chien KS, Hammond D, Montalban-Bravo G, Borthakur G, Short N, Issa GC, Kadia TM, Daver N, Tang G, Quesada A, Patel KP, Ravandi F, Fiskus W, Mill CP, Kantarjian HM, Bhalla K, Garcia-Manero G, Oran B, DiNardo CD
Am J Hematol 2023 Nov;98(11):1780-1790. Epub 2023 Sep 4 doi: 10.1002/ajh.27070. PMID: 37665752Free PMC Article
Childhood leukemia.
Hutter JJ
Pediatr Rev 2010 Jun;31(6):234-41. doi: 10.1542/pir.31-6-234. PMID: 20516235
Chronic myelomonocytic leukemia.
Bennett JM
Curr Treat Options Oncol 2002 Jun;3(3):221-3. doi: 10.1007/s11864-002-0011-6. PMID: 12057067
Juvenile myelomonocytic leukemia.
Aricò M, Biondi A, Pui CH
Blood 1997 Jul 15;90(2):479-88. PMID: 9226148

Prognosis

How I diagnose and treat chronic myelomonocytic leukemia.
Patnaik MM
Haematologica 2022 Jul 1;107(7):1503-1517. doi: 10.3324/haematol.2021.279500. PMID: 35236051Free PMC Article
Chronic myelomonocytic leukemia: 2022 update on diagnosis, risk stratification, and management.
Patnaik MM, Tefferi A
Am J Hematol 2022 Mar 1;97(3):352-372. Epub 2022 Jan 27 doi: 10.1002/ajh.26455. PMID: 34985762
Myelodysplastic/myeloproliferative neoplasms.
Cazzola M, Malcovati L, Invernizzi R
Hematology Am Soc Hematol Educ Program 2011;2011:264-72. doi: 10.1182/asheducation-2011.1.264. PMID: 22160044
Childhood leukemia.
Hutter JJ
Pediatr Rev 2010 Jun;31(6):234-41. doi: 10.1542/pir.31-6-234. PMID: 20516235
Juvenile myelomonocytic leukemia.
Aricò M, Biondi A, Pui CH
Blood 1997 Jul 15;90(2):479-88. PMID: 9226148

Clinical prediction guides

Technical, gating and interpretation recommendations for the partitioning of circulating monocyte subsets assessed by flow cytometry.
Tarfi S, Kern W, Goulas E, Selimoglu-Buet D, Wagner-Ballon O; CytHem‐LMMC
Cytometry B Clin Cytom 2024 May;106(3):203-215. Epub 2024 Apr 24 doi: 10.1002/cyto.b.22176. PMID: 38656036
How I diagnose and treat chronic myelomonocytic leukemia.
Patnaik MM
Haematologica 2022 Jul 1;107(7):1503-1517. doi: 10.3324/haematol.2021.279500. PMID: 35236051Free PMC Article
Genetic complexity of chronic myelomonocytic leukemia.
Patel AB, Deininger MW
Leuk Lymphoma 2021 May;62(5):1031-1045. Epub 2020 Dec 18 doi: 10.1080/10428194.2020.1856837. PMID: 33337259
Role of RUNX1 in hematological malignancies.
Sood R, Kamikubo Y, Liu P
Blood 2017 Apr 13;129(15):2070-2082. Epub 2017 Feb 8 doi: 10.1182/blood-2016-10-687830. PMID: 28179279Free PMC Article
Allogeneic hematopoietic stem cell transplantation for MDS and CMML: recommendations from an international expert panel.
de Witte T, Bowen D, Robin M, Malcovati L, Niederwieser D, Yakoub-Agha I, Mufti GJ, Fenaux P, Sanz G, Martino R, Alessandrino EP, Onida F, Symeonidis A, Passweg J, Kobbe G, Ganser A, Platzbecker U, Finke J, van Gelder M, van de Loosdrecht AA, Ljungman P, Stauder R, Volin L, Deeg HJ, Cutler C, Saber W, Champlin R, Giralt S, Anasetti C, Kröger N
Blood 2017 Mar 30;129(13):1753-1762. Epub 2017 Jan 17 doi: 10.1182/blood-2016-06-724500. PMID: 28096091Free PMC Article

Recent systematic reviews

Investigating historic cases of pyoderma gangrenosum in myelodysplastic syndrome and chronic myelomonocytic leukemia for possible VEXAS syndrome: A systematic review.
Croitoru DO, Huang RS, McMullen EP, Maazi M, Piguet V
J Am Acad Dermatol 2024 Oct;91(4):712-714. Epub 2024 Jun 19 doi: 10.1016/j.jaad.2024.05.086. PMID: 38906261
CSF3R-mutant chronic myelomonocytic leukemia is a distinct clinically subset with abysmal prognosis: a case report and systematic review of the literature.
Guastafierro V, Ubezio M, Manes N, Milanesi C, Della Porta M, Bonometti A
Leuk Lymphoma 2023 Sep;64(9):1566-1573. Epub 2023 Jul 3 doi: 10.1080/10428194.2023.2227750. PMID: 37395413
Hypomethylating agents in the treatment of chronic myelomonocytic leukemia: a meta-analysis and systematic review.
Xu R, Li M, Wu P, Deng C, Geng S, Huang X, Weng J, Du X
Hematology 2021 Dec;26(1):312-320. doi: 10.1080/16078454.2021.1875600. PMID: 33706667
A systematic review and meta-analysis of the efficacy and adverse events of azacitidine-plus-lenalidomide treatment for patients with acute myeloid leukemia, myelodysplastic syndromes and chronic myelomonocytic leukemia (1).
Kunacheewa C, Thongthang P, Ungprasert P, Utchariyaprasit E, Owattanapanich W
Hematology 2019 Dec;24(1):498-506. doi: 10.1080/16078454.2019.1631425. PMID: 31221030
Systematic Literature Review of Treatment Options and Clinical Outcomes for Patients With Higher-Risk Myelodysplastic Syndromes and Chronic Myelomonocytic Leukemia.
Bell JA, Galaznik A, Huelin R, Stokes M, Guo Y, Fram RJ, Faller DV
Clin Lymphoma Myeloma Leuk 2018 Apr;18(4):e157-e166. Epub 2018 Feb 8 doi: 10.1016/j.clml.2018.02.001. PMID: 29475821

Supplemental Content

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    Clinical resources

    Practice guidelines

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    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Myelodysplastic Syndromes, 2024

    Reviews

    Related information

    • ClinVar
      Related medical variations
    • GTR
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    • GTR(Clinical)
      Clinical tests in GTR
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      Related Medical Subject Headings
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      Related records in OMIM
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