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      KCI등재 SCOPUS

      Diagnosis and treatment of chronic myelomonocytic leukemia

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      https://www.riss.kr/link?id=A107384891

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      다국어 초록 (Multilingual Abstract)

      Chronic myelomonocytic leukemia (CMML) is a clonal disorder of hematopoietic cells and is a complex of heterogeneous conditions with both myeloproliferative and myelodysplastic features. The diagnosis of CMML is made using morphologic criteria includi...

      Chronic myelomonocytic leukemia (CMML) is a clonal disorder of hematopoietic cells and is a complex of heterogeneous conditions with both myeloproliferative and myelodysplastic features. The diagnosis of CMML is made using morphologic criteria including monocyte-dominant leukocytosis, dysplastic changes, and increased blasts in the bone marrow. Recently, the identification of monocyte subtypes in peripheral blood using multiparameter flow cytometry has been actively studied. Chromosomal abnormalities are the basis of CMML risk stratification, and mutations in several genes including ASXL1 are known to be important not only for the diagnosis and treatment of this disease but also for predicting its prognosis. The standard treatment principles for CMML have not yet been clearly defined; however, hypomethylating agents are mainly considered the frontline therapy in most cases. Although allogeneic hematopoietic stem cell transplantation has limited applications owing to its toxicity, it still plays an important role as the only curative treatment option. Researchers are continuing to develop new drugs for CMML treatment and to prove their clinical usefulness. This review summarizes what is known to date on the diagnosis, treatment, and prognostic factors of CMML and presents future directions by analyzing recent research trends.

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      참고문헌 (Reference)

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      1 Ball M, "When clinical heterogeneity exceeds genetic heterogeneity : thinking outside the genomic box in chronic myelomonocytic leukemia" 128 : 2381-2387, 2016

      2 Kongtim P, "Treatment with hypomethylating agents before allogeneic stem cell transplant improves progression-free survival for patients with chronic myelomonocytic leukemia" 22 : 47-53, 2016

      3 Santini V, "Treatment of low-risk myelodysplastic syndromes" 2016 : 462-469, 2016

      4 Wong E, "Treatment of chronic myelomonocytic leukemia with azacitidine" 54 : 878-880, 2013

      5 Thorpe M, "Treatment of chronic myelomonocytic leukemia with 5-Azacitidine : a case series and literature review" 36 : 1071-1073, 2012

      6 Cargo C, "The use of targeted sequencing and flow cytometry to identify patients with a clinically significant monocytosis" 133 : 1325-1334, 2019

      7 Dinmohamed AG, "The use of medical claims to assess incidence, diagnostic procedures and initial treatment of myelodysplastic syndromes and chronic myelomonocytic leukemia in the Netherlands" 39 : 177-182, 2015

      8 Bennett JM, "The chronic myeloid leukaemias: guidelines for distinguishing chronic granulocytic, atypical chronic myeloid, and chronic myelomonocytic leukaemia. Proposals by the French–American–British Cooperative Leukaemia Group" 87 : 746-754, 1994

      9 Arber DA, "The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia" 127 : 2391-2405, 2016

      10 Patnaik MM, "Targeted next generation sequencing of PDGFRB rearranged myeloid neoplasms with monocytosis" 91 : E12-E14, 2016

      11 Coston T, "Suboptimal response rates to hypomethylating agent therapy in chronic myelomonocytic leukemia; a single institutional study of 121 patients" 94 : 767-779, 2019

      12 Laborde RR, "SETBP1 mutations in 415 patients with primary myelofibrosis or chronic myelomonocytic leukemia : independent prognostic impact in CMML" 27 : 2100-2102, 2013

      13 Steensma DP, "Results of a clinical trial of H3B-8800, a splicing modulator, in patients with myelodysplastic syndromes(MDS), acute myeloid leukemia(AML)or chronic myelomonocytic leukemia(CMML)" 134 (134): 673-, 2019

      14 Patnaik MM, "Results from ongoing phase 1/2clinical trial of tagraxofusp(SL-401)in patients with relapsed/refractory chronic myelomonocytic leukemia(CMML)" 37 (37): 7059-7059, 2019

      15 Xicoy B, "Response to erythropoieticstimulating agents in patients with chronic myelomonocytic leukemia" 97 : 33-38, 2016

      16 Fenaux P, "Randomized, double-blind, placebo-controlled, multicenter study evaluating epoetin alfa versus placebo in anemic patients with IPSS low-INT1risk MDS" P248-, 2016

      17 Sekeres MA, "Randomized phase II study of azacitidine alone or in combination with lenalidomide or with vorinostat in higher-risk myelodysplastic syndromes and chronic myelomonocytic leukemia : North American Intergroup Study SWOG S1117" 35 : 2745-2753, 2017

      18 Silverman LR, "Randomized controlled trial of azacitidine in patients with the myelodysplastic syndrome : a study of the cancer and leukemia group B" 20 : 2429-2440, 2002

      19 Kuo MC, "RUNX1 mutations are frequent in chronic myelomonocytic leukemia and mutations at the C-terminal region might predict acute myeloid leukemia transformation" 23 : 1426-1431, 2009

      20 Ricci C, "RAS mutations contribute to evolution of chronic myelomonocytic leukemia to the proliferative variant" 16 : 2246-2256, 2010

      21 Valent P, "Proposed diagnostic criteria for classical chronic myelomonocytic leukemia(CMML), CMML variants and pre-CMML conditions" 104 : 1935-1949, 2019

      22 Kantarjian H, "Proposal for a new risk model in myelodysplastic syndrome that accounts for events not considered in the original International Prognostic Scoring System" 113 : 1351-1361, 2008

      23 Padron E, "Promising results of a phase 1/2 clinical trial of ruxolitinib in patients with chronic myelomonocytic leukemia" 130 (130): 162-162, 2017

      24 Itzykson R, "Prognostic score including gene mutations in chronic myelomonocytic leukemia" 31 : 2428-2436, 2013

      25 Duchmann M, "Prognostic role of gene mutations in chronic myelomonocytic leukemia patients treated with hypomethylating agents" 31 : 174-181, 2018

      26 Patnaik MM, "Prognostic interaction between ASXL1 and TET2 mutations in chronic myelomonocytic leukemia" 6 : e385-, 2016

      27 Tang G, "Prognostic impact of acquisition of cytogenetic abnormalities during the course of chronic myelomonocytic leukemia" 90 : 882-887, 2015

      28 Onida F, "Prognostic factors and scoring systems in chronic myelomonocytic leukemia : a retrospective analysis of 213 patients" 99 : 840-849, 2002

      29 Patnaik MM, "Preliminary results from an open-label, phase 2 study of tipifarnib in chronic myelomonocytic leukemia(CMML)" (130) : 2963-2963, 2017

      30 Adès L, "Predictive factors of response and survival among chronic myelomonocytic leukemia patients treated with azacitidine" 37 : 609-613, 2013

      31 Oganesian A, "Preclinical data in cynomolgus(cyn)monkeys of ASTX727, a novel oral hypomethylating agent(HMA)composed of low-dose oral decitabine combined with a novel cytidine deaminase inhibitor(CDAi)E7727" 122 (122): 2526-2526, 2013

      32 Pophali PA, "Practical limitations of monocyte subset repartitioning by multiparametric flow cytometry in chronic myelomonocytic leukemia" 9 : 65-, 2019

      33 Garcia-Manero G, "Phase I study of oral azacitidine in myelodysplastic syndromes, chronic myelomonocytic leukemia, and acute myeloid leukemia" 29 : 2521-2527, 2011

      34 Patnaik MM, "Phase 1 study of lenzilumab, a recombinant anti-human GM-CSF antibody, for chronic myelomonocytic leukemia" 136 : 909-913, 2020

      35 Garcia-Manero G, "Pharmacokinetic exposure equivalence and preliminary efficacy and safety from a randomized cross over phase 3 study(ASCERTAIN study)of an oral hypomethylating agent ASTX727(cedazuridine/decitabine)compared to IV decitabine" 134 (134): 846-846, 2019

      36 Ma Y, "Pacritinib(PAC)synergistically potentiates azacitidine(5AZA)cytotoxicity in chronic myelomonocytic leukemia(CMML)" 126 (126): 1658-1658, 2015

      37 Ocheni S, "Outcome of allo-SCT for chronic myelomonocytic leukemia" 43 : 659-661, 2009

      38 Garcia-Manero G, "Oral cedazuridine/decitabine for MDS and CMML : a phase 2pharmacokinetic/pharmacodynamic randomized crossover study" 136 : 674-683, 2020

      39 Geyer JT, "Oligomonocytic chronic myelomonocytic leukemia(chronic myelomonocytic leukemia without absolute monocytosis)displays a similar clinicopathologic and mutational profile to classical chronic myelomonocytic leukemia" 30 : 1213-1222, 2017

      40 Ziegler-Heitbrock L, "Nomenclature of monocytes and dendritic cells in blood" 116 : e74-e80, 2010

      41 Kohlmann A, "Next-generation sequencing technology reveals a characteristic pattern of molecular mutations in 72.8% of chronic myelomonocytic leukemia by detecting frequent alterations in TET2, CBL, RAS, and RUNX1" 28 : 3858-3865, 2010

      42 Alfonso A, "Natural history of chronic myelomonocytic leukemia treated with hypomethylating agents" 92 : 599-606, 2017

      43 Schuler E, "Myelodysplastic syndromes without peripheral monocytosis but with evidence of marrow monocytosis share clinical and molecular characteristics with CMML" 65 : 1-4, 2018

      44 Jankowska AM, "Mutational spectrum analysis of chronic myelomonocytic leukemia includes genes associated with epigenetic regulation : UTX, EZH2, and DNMT3A" 118 : 3932-3941, 2011

      45 Merlevede J, "Mutation allele burden remains unchanged in chronic myelomonocytic leukaemia responding to hypomethylating agents" 7 : 10767-, 2016

      46 Tarfi S, "Multicentric validation of the"monocyte assay"for chronic myelomonocytic leukemia diagnosis by flow cytometry" 130 (130): 4273-4273, 2017

      47 Talati C, "Monocyte subset analysis accurately distinguishes CMML from MDS and is associated with a favorable MDS prognosis" 129 : 1881-1883, 2017

      48 Grossmann V, "Molecular profiling of chronic myelomonocytic leukemia reveals diverse mutations in >80% of patients with TET2 and EZH2 being of high prognostic relevance" 25 : 877-879, 2011

      49 Braun T, "Molecular predictors of response to decitabine in advanced chronic myelomonocytic leukemia : a phase 2 trial" 118 : 3824-3831, 2011

      50 Palomo L, "Molecular landscape and clonal architecture of adult myelodysplastic/myeloproliferative neoplasms" 136 : 1851-1862, 2020

      51 Wassie EA, "Molecular and prognostic correlates of cytogenetic abnormalities in chronic myelomonocytic leukemia : a Mayo Clinic–French Consortium Study" 89 : 1111-1115, 2014

      52 Patnaik MM, "Mayo prognostic model for WHO-defined chronic myelomonocytic leukemia : ASXL1and spliceosome component mutations and outcomes" 27 : 1504-1510, 2013

      53 Schmid C, "Long-term survival in refractory acute myeloid leukemia after sequential treatment with chemotherapy and reduced-intensity conditioning for allogeneic stem cell transplantation" 108 : 1092-1099, 2006

      54 Buradkar A, "Landscape of RAS pathway mutations in patients with myelodysplastic syndrome/myeloproliferative neoplasm overlap syndromes : a study of 461 molecularly annotated patients" 35 : 644-649, 2021

      55 Elena C, "Integrating clinical features and genetic lesions in the risk assessment of patients with chronic myelomonocytic leukemia" 128 : 1408-1417, 2016

      56 Neukirchen J, "Incidence and prevalence of myelodysplastic syndromes : data from the Düsseldorf MDS-registry" 35 : 1591-1596, 2011

      57 Subari S, "Hypomethylating agents are effective in shrinking splenomegaly in patients with chronic myelomonocytic leukemia" 57 : 1714-1715, 2016

      58 Fianchi L, "High rate of remissions in chronic myelomonocytic leukemia treated with 5-azacytidine : results of an Italian retrospective study" 54 : 658-661, 2013

      59 Seiler M, "H3B-8800, an orally available small-molecule splicing modulator, induces lethality in spliceosome-mutant cancers" 24 : 497-504, 2018

      60 Patel BJ, "Genomic determinants of chronic myelomonocytic leukemia" 31 : 2815-2823, 2017

      61 Padron E, "GM-CSF-dependent pSTAT5sensitivity is a feature with therapeutic potential in chronic myelomonocytic leukemia" 121 : 5068-5077, 2013

      62 Wijermans PW, "Efficacy of decitabine in the treatment of patients with chronic myelomonocytic leukemia(CMML)" 32 : 587-591, 2008

      63 Fenaux P, "Efficacy of azacitidine compared with that of conventional care regimens in the treatment of higher-risk myelodysplastic syndromes: a randomised, open-label, phase III study" 10 : 223-232, 2009

      64 Patnaik MM, "EZH2 mutations in chronic myelomonocytic leukemia cluster with ASXL1mutations and their co-occurrence is prognostically detrimental" 8 : 12-, 2018

      65 Itzykson R, "Diagnosis and treatment of chronic myelomonocytic leukemias in adults : recommendations from the European Hematology Association and the European LeukemiaNet" 2 : e150-, 2018

      66 Döhner H, "Diagnosis and management of AML in adults : 2017 ELN recommendations from an international expert panel" 129 : 424-447, 2017

      67 Such E, "Development and validation of a prognostic scoring system for patients with chronic myelomonocytic leukemia" 121 : 3005-3015, 2013

      68 Bell GC, "Detection of a PDGFRB fusion in refractory CMML without eosinophilia : a case for broad spectrum tumor profiling" 4 : 70-71, 2015

      69 Dhillon S, "Decitabine/cedazuridine : first approval" 80 : 1373-1378, 2020

      70 Patnaik MM, "DNMT3A mutations are associated with inferior overall and leukemia-free survival in chronic myelomonocytic leukemia" 92 : 56-61, 2017

      71 Hunter AM, "Current management and recent advances in the treatment of chronic myelomonocytic leukemia" 19 : 67-, 2018

      72 Kunimoto H, "Cooperative epigenetic remodeling by TET2 loss and NRAS mutation drives myeloid transformation and MEK inhibitor sensitivity" 33 : 44-59, 2018

      73 Abdel-Wahab O, "Concomitant analysis of EZH2 and ASXL1 mutations in myelofibrosis, chronic myelomonocytic leukemia and blast-phase myeloproliferative neoplasms" 25 : 1200-1202, 2011

      74 Geissler K, "Chronic myelomonocytic leukemia patients with RAS pathway mutations show high in vitro myeloid colony formation in the absence of exogenous growth factors" 30 : 2280-2281, 2016

      75 Patnaik MM, "Chronic myelomonocytic leukemia : 2020update on diagnosis, risk stratification and management" 95 : 97-115, 2020

      76 Patnaik MM, "Chronic myelomonocytic leukaemia : a concise clinical and pathophysiological review" 165 : 273-286, 2014

      77 Selimoglu-Buet D, "Characteristic repartition of monocyte subsets as a diagnostic signature of chronic myelomonocytic leukemia" 125 : 3618-3626, 2015

      78 Lucas N, "Biology and prognostic impact of clonal plasmacytoid dendritic cells in chronic myelomonocytic leukemia" 33 : 2466-2480, 2019

      79 Pleyer L, "Azacitidine in CMML : matched-pair analyses of daily-life patients reveal modest effects on clinical course and survival" 38 : 475-483, 2014

      80 Kröger N, "Allogeneic stem cell transplantation of adult chronic myelomonocytic leukaemia. A report on behalf of the Chronic Leukaemia Working Party of the European Group for Blood and Marrow Transplantation (EBMT)" 118 : 67-73, 2002

      81 Park S, "Allogeneic stem cell transplantation for chronic myelomonocytic leukemia : a report from the Societe Francaise de Greffe de Moelle et de Therapie Cellulaire" 90 : 355-364, 2013

      82 Elliott MA, "Allogeneic stem cell transplantation and donor lymphocyte infusions for chronic myelomonocytic leukemia" 37 : 1003-1008, 2006

      83 Eissa H, "Allogeneic hematopoietic cell transplantation for chronic myelomonocytic leukemia : relapse-free survival is determined by karyotype and comorbidities" 17 : 908-915, 2011

      84 Kerbauy DM, "Allogeneic hematopoietic cell transplantation for chronic myelomonocytic leukemia" 11 : 713-720, 2005

      85 Krishnamurthy P, "Allogeneic haematopoietic SCT for chronic myelomonocytic leukaemia : a single-centre experience" 45 : 1502-1507, 2010

      86 Mason CC, "Age-related mutations and chronic myelomonocytic leukemia" 30 : 906-913, 2016

      87 Borthakur G, "Activity of the oral mitogen-activated protein kinase kinase inhibitor trametinib in RAS-mutant relapsed or refractory myeloid malignancies" 122 : 1871-1879, 2016

      88 Aribi A, "Activity of decitabine, a hypomethylating agent, in chronic myelomonocytic leukemia" 109 : 713-717, 2007

      89 Costa R, "Activity of azacitidine in chronic myelomonocytic leukemia" 117 : 2690-2696, 2011

      90 Symeonidis A, "Achievement of complete remission predicts outcome of allogeneic haematopoietic stem cell transplantation in patients with chronic myelomonocytic leukaemia. A study of the Chronic Malignancies Working Party of the European Group for Blood and Marrow Transplantation" 171 : 239-246, 2015

      91 Gelsi-Boyer V, "ASXL1 mutation is associated with poor prognosis and acute transformation in chronic myelomonocytic leukaemia" 151 : 365-375, 2010

      92 Patnaik MM, "ASXL1 and SETBP1mutations and their prognostic contribution in chronic myelomonocytic leukemia : a two-center study of 466 patients" 28 : 2206-2212, 2014

      93 Platzbecker U, "ARCADE (20090160): a phase 3 randomized placebo-controlled double-blind trial of darbepoetin alpha in the treatment of anemia in patients with low or intermediate-1 risk myelodysplastic syndromes (MDS)" S128-, 2016

      94 Wattel E, "A randomized trial of hydroxyurea versus VP16 in adult chronic myelomonocytic leukemia. Groupe Francais des Myelodysplasies and European CMML Group" 88 : 2480-2487, 1996

      95 Santini V, "A phase II, multicentre trial of decitabine in higher-risk chronic myelomonocytic leukemia" 32 : 413-418, 2018

      96 Tantravahi SK, "A phase II study of the efficacy, safety, and determinants of response to 5-azacitidine(VidazaⓇ)in patients with chronic myelomonocytic leukemia" 57 : 2441-2444, 2016

      97 Fenaux P, "A multicenter phase 2 study of the farnesyltransferase inhibitor tipifarnib in intermediate-to high-risk myelodysplastic syndrome" 109 : 4158-4163, 2007

      98 Drummond MW, "A multi-centre phase 2 study of azacitidine in chronic myelomonocytic leukaemia" 28 : 1570-1572, 2014

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      2023 평가예정 해외DB학술지평가 신청대상 (해외등재 학술지 평가)
      2020-01-01 평가 등재학술지 유지 (해외등재 학술지 평가) KCI등재
      2013-11-22 학술지명변경 한글명 : 대한혈액학회지 -> Blood Research
      외국어명 : The Korean Journal of Hematology -> Blood Research
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      2012-02-01 평가 SCOPUS 등재 (등재유지) KCI등재
      2011-01-01 평가 등재학술지 유지 (등재유지) KCI등재
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      2007-01-01 평가 등재학술지 유지 (등재유지) KCI등재
      2006-04-06 학술지명변경 외국어명 : 미등록 -> The Korean Journal of Hematology KCI등재
      2004-01-01 평가 등재학술지 선정 (등재후보2차) KCI등재
      2003-01-01 평가 등재후보 1차 PASS (등재후보1차) KCI등재후보
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