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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.
 
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.

Professional guidelines

PubMed

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
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
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

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
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
Patnaik MM
Haematologica 2022 Jul 1;107(7):1503-1517. doi: 10.3324/haematol.2021.279500. PMID: 35236051Free PMC Article
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
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

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
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
Patnaik MM
Haematologica 2022 Jul 1;107(7):1503-1517. doi: 10.3324/haematol.2021.279500. PMID: 35236051Free PMC Article
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
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

Therapy

Itzykson R, Santini V, Thepot S, Ades L, Chaffaut C, Giagounidis A, Morabito M, Droin N, Lübbert M, Sapena R, Nimubona S, Goasguen J, Wattel E, Zini G, Torregrosa Diaz JM, Germing U, Pelizzari AM, Park S, Jaekel N, Metzgeroth G, Onida F, Navarro R, Patriarca A, Stamatoullas A, Götze K, Puttrich M, Mossuto S, Solary E, Gloaguen S, Chevret S, Chermat F, Platzbecker U, Fenaux P
J Clin Oncol 2023 Apr 1;41(10):1888-1897. Epub 2022 Dec 1 doi: 10.1200/JCO.22.00437. PMID: 36455187
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
Da Costa EM, McInnes G, Beaudry A, Raynal NJ
Cancer J 2017 Sep/Oct;23(5):270-276. doi: 10.1097/PPO.0000000000000278. PMID: 28926427
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
Selimoglu-Buet D, Wagner-Ballon O, Saada V, Bardet V, Itzykson R, Bencheikh L, Morabito M, Met E, Debord C, Benayoun E, Nloga AM, Fenaux P, Braun T, Willekens C, Quesnel B, Adès L, Fontenay M, Rameau P, Droin N, Koscielny S, Solary E; Francophone Myelodysplasia Group
Blood 2015 Jun 4;125(23):3618-26. Epub 2015 Apr 7 doi: 10.1182/blood-2015-01-620781. PMID: 25852055Free PMC Article

Prognosis

Patnaik MM
Haematologica 2022 Jul 1;107(7):1503-1517. doi: 10.3324/haematol.2021.279500. PMID: 35236051Free PMC Article
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
Belotserkovskaya E, Demidov O
Int J Mol Sci 2021 Oct 26;22(21) doi: 10.3390/ijms222111510. PMID: 34768940Free PMC Article
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
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

Clinical prediction guides

Patnaik MM
Haematologica 2022 Jul 1;107(7):1503-1517. doi: 10.3324/haematol.2021.279500. PMID: 35236051Free PMC Article
Palomo L, Meggendorfer M, Hutter S, Twardziok S, Ademà V, Fuhrmann I, Fuster-Tormo F, Xicoy B, Zamora L, Acha P, Kerr CM, Kern W, Maciejewski JP, Solé F, Haferlach C, Haferlach T
Blood 2020 Oct 15;136(16):1851-1862. doi: 10.1182/blood.2019004229. PMID: 32573691Free PMC Article
Garcia-Manero G, Griffiths EA, Steensma DP, Roboz GJ, Wells R, McCloskey J, Odenike O, DeZern AE, Yee K, Busque L, O'Connell C, Michaelis LC, Brandwein J, Kantarjian H, Oganesian A, Azab M, Savona MR
Blood 2020 Aug 6;136(6):674-683. doi: 10.1182/blood.2019004143. PMID: 32285126Free PMC Article
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
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

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
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
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
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
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

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.
    • Bookshelf
      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

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