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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 update on hypomethylating agents.
Duchmann M, Itzykson R
Int J Hematol 2019 Aug;110(2):161-169. Epub 2019 Apr 24 doi: 10.1007/s12185-019-02651-9. PMID: 31020568

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
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
Myelodysplastic/myeloproliferative neoplasms: a disease in need of recognition.
Khan M, Sarfraz M, Daver N
Future Oncol 2017 Jan;13(2):117-120. Epub 2016 Sep 27 doi: 10.2217/fon-2016-0310. PMID: 27676208

Therapy

Chronic myelomonocytic leukaemia causing orbital inflammation.
McGrath R, Fay M, McAnena L
BMJ Case Rep 2024 Oct 30;17(10) doi: 10.1136/bcr-2023-258203. PMID: 39477459
Phase to phase: Navigating drug combinations with hypomethylating agents in higher-risk MDS trials for optimal outcomes.
Merz AMA, Sébert M, Sonntag J, Kubasch AS, Platzbecker U, Adès L
Cancer Treat Rev 2024 Feb;123:102673. Epub 2023 Dec 19 doi: 10.1016/j.ctrv.2023.102673. PMID: 38176221
Clinical update on hypomethylating agents.
Duchmann M, Itzykson R
Int J Hematol 2019 Aug;110(2):161-169. Epub 2019 Apr 24 doi: 10.1007/s12185-019-02651-9. PMID: 31020568
Myelodysplastic/myeloproliferative neoplasms: a disease in need of recognition.
Khan M, Sarfraz M, Daver N
Future Oncol 2017 Jan;13(2):117-120. Epub 2016 Sep 27 doi: 10.2217/fon-2016-0310. PMID: 27676208
Childhood leukemia.
Hutter JJ
Pediatr Rev 2010 Jun;31(6):234-41. doi: 10.1542/pir.31-6-234. PMID: 20516235

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
Autoimmune disease in CMML-the chicken or the egg?
Ambinder AJ, Miller J, DeZern AE
Best Pract Res Clin Haematol 2020 Jun;33(2):101136. Epub 2019 Dec 17 doi: 10.1016/j.beha.2019.101136. PMID: 32460986
Assessing Symptom Burden in Myelodysplastic Syndrome/Myeloproliferative Neoplasm Overlap Patients.
Garza J, Anderson JM, Scherber RM
Hematol Oncol Clin North Am 2020 Apr;34(2):475-489. Epub 2019 Dec 25 doi: 10.1016/j.hoc.2019.11.001. PMID: 32089224
Chronic myelomoncytic leukemia: Are we finally solving the identity crisis?
Nazha A, Prebet T, Gore S, Zeidan AM
Blood Rev 2016 Sep;30(5):381-8. Epub 2016 Apr 25 doi: 10.1016/j.blre.2016.04.006. PMID: 27162201

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
A three-gene leukaemic stem cell signature score is robustly prognostic in chronic myelomonocytic leukaemia.
Wang YH, Yao CY, Lin CC, Gurashi K, Amaral FMR, Bossenbroek H, Jerez A, Somervaille TCP, Binder M, Patnaik MM, Hou HA, Chou WC, Batta K, Wiseman DH, Tien HF
Br J Haematol 2023 Apr;201(2):302-307. Epub 2023 Feb 6 doi: 10.1111/bjh.18681. PMID: 36746431
Monocytosis in primary care and risk of haematological malignancies.
Christensen ME, Siersma V, Kriegbaum M, Lind BS, Samuelsson J, Østgård LSG, Grønbaek K, Andersen CL
Eur J Haematol 2023 Apr;110(4):362-370. Epub 2022 Dec 16 doi: 10.1111/ejh.13911. PMID: 36479724
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
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

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
Cutaneous involvement in Ph-negative myeloproliferative neoplasms: from extramedullary hematopoiesis to myeloid metastasis with histiocytic differentiation. A systematic review of the literature.
Bonometti A
Int J Dermatol 2023 Oct;62(10):1228-1236. Epub 2023 Aug 30 doi: 10.1111/ijd.16809. PMID: 37649236
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
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

Table of contents

    Clinical resources

    Practice guidelines

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

    Curated

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

    Reviews

    Related information

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