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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]

Professional guidelines

PubMed

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
Chronic myelomonocytic leukemia diagnosis and management.
Chan O, Renneville A, Padron E
Leukemia 2021 Jun;35(6):1552-1562. Epub 2021 Mar 13 doi: 10.1038/s41375-021-01207-3. PMID: 33714974
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

Recent clinical studies

Etiology

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
Chronic myelomonocytic leukemia diagnosis and management.
Chan O, Renneville A, Padron E
Leukemia 2021 Jun;35(6):1552-1562. Epub 2021 Mar 13 doi: 10.1038/s41375-021-01207-3. PMID: 33714974
Chronic myelomonocytic leukemia - a review.
Thomopoulos TP, Bouhla A, Papageorgiou SG, Pappa V
Expert Rev Hematol 2021 Jan;14(1):59-77. Epub 2020 Dec 12 doi: 10.1080/17474086.2021.1860004. PMID: 33275852

Diagnosis

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
Chronic myelomonocytic leukemia diagnosis and management.
Chan O, Renneville A, Padron E
Leukemia 2021 Jun;35(6):1552-1562. Epub 2021 Mar 13 doi: 10.1038/s41375-021-01207-3. PMID: 33714974
Chronic myelomonocytic leukemia - a review.
Thomopoulos TP, Bouhla A, Papageorgiou SG, Pappa V
Expert Rev Hematol 2021 Jan;14(1):59-77. Epub 2020 Dec 12 doi: 10.1080/17474086.2021.1860004. PMID: 33275852

Therapy

Pevonedistat plus azacitidine vs azacitidine alone in higher-risk MDS/chronic myelomonocytic leukemia or low-blast-percentage AML.
Adès L, Girshova L, Doronin VA, Díez-Campelo M, Valcárcel D, Kambhampati S, Viniou NA, Woszczyk D, De Paz Arias R, Symeonidis A, Anagnostopoulos A, Munhoz EC, Platzbecker U, Santini V, Fram RJ, Yuan Y, Friedlander S, Faller DV, Sekeres MA
Blood Adv 2022 Sep 13;6(17):5132-5145. doi: 10.1182/bloodadvances.2022007334. PMID: 35728048Free PMC Article
Role of allogeneic transplantation in chronic myelomonocytic leukemia: an international collaborative analysis.
Robin M, de Wreede LC, Padron E, Bakunina K, Fenaux P, Koster L, Nazha A, Beelen DW, Rampal RK, Sockel K, Komrokji RS, Gagelmann N, Eikema DJ, Radujkovic A, Finke J, Potter V, Killick SB, Legrand F, Solary E, Broom A, Garcia-Manero G, Rizzoli V, Hayden P, Patnaik MM, Onida F, Yakoub-Agha I, Itzykson R
Blood 2022 Sep 22;140(12):1408-1418. doi: 10.1182/blood.2021015173. PMID: 35667047
Chronic myelomonocytic leukemia diagnosis and management.
Chan O, Renneville A, Padron E
Leukemia 2021 Jun;35(6):1552-1562. Epub 2021 Mar 13 doi: 10.1038/s41375-021-01207-3. PMID: 33714974
Myelodysplastic syndrome/myeloproliferative neoplasm overlap syndromes: a focused review.
Patnaik MM, Lasho T
Hematology Am Soc Hematol Educ Program 2020 Dec 4;2020(1):460-464. doi: 10.1182/hematology.2020000163. PMID: 33275673Free PMC Article
Decitabine/Cedazuridine: First Approval.
Dhillon S
Drugs 2020 Sep;80(13):1373-1378. doi: 10.1007/s40265-020-01389-7. PMID: 32860582Free PMC Article

Prognosis

Role of allogeneic transplantation in chronic myelomonocytic leukemia: an international collaborative analysis.
Robin M, de Wreede LC, Padron E, Bakunina K, Fenaux P, Koster L, Nazha A, Beelen DW, Rampal RK, Sockel K, Komrokji RS, Gagelmann N, Eikema DJ, Radujkovic A, Finke J, Potter V, Killick SB, Legrand F, Solary E, Broom A, Garcia-Manero G, Rizzoli V, Hayden P, Patnaik MM, Onida F, Yakoub-Agha I, Itzykson R
Blood 2022 Sep 22;140(12):1408-1418. doi: 10.1182/blood.2021015173. PMID: 35667047
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
Chronic myelomonocytic leukemia diagnosis and management.
Chan O, Renneville A, Padron E
Leukemia 2021 Jun;35(6):1552-1562. Epub 2021 Mar 13 doi: 10.1038/s41375-021-01207-3. PMID: 33714974
Chronic myelomonocytic leukemia - a review.
Thomopoulos TP, Bouhla A, Papageorgiou SG, Pappa V
Expert Rev Hematol 2021 Jan;14(1):59-77. Epub 2020 Dec 12 doi: 10.1080/17474086.2021.1860004. PMID: 33275852

Clinical prediction guides

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
Myeloproliferative Neoplasms with Monocytosis.
Morsia E, Gangat N
Curr Hematol Malig Rep 2022 Feb;17(1):46-51. Epub 2021 Nov 13 doi: 10.1007/s11899-021-00660-2. PMID: 34773576
Chronic myelomonocytic leukemia diagnosis and management.
Chan O, Renneville A, Padron E
Leukemia 2021 Jun;35(6):1552-1562. Epub 2021 Mar 13 doi: 10.1038/s41375-021-01207-3. PMID: 33714974
Decitabine/Cedazuridine: First Approval.
Dhillon S
Drugs 2020 Sep;80(13):1373-1378. doi: 10.1007/s40265-020-01389-7. PMID: 32860582Free 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

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
Myeloid neoplasms with t(12;22)(p13;q12)/MN1-EVT6: a systematic review of 12 cases.
Shao H, Cen J, Chen S, Qiu H, Pan J
Ann Hematol 2018 Mar;97(3):417-424. Epub 2017 Dec 22 doi: 10.1007/s00277-017-3208-2. PMID: 29273914

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