Chronic myelomonocytic leukemia: 2016 update on diagnosis, risk stratification, and management

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Abstract

Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic stem cell disorder characterized by overlapping features of myelodysplastic syndromes and myeloproliferative neoplasms. Diagnosis is based on the presence of persistent (>3months) peripheral blood monocytosis (>1×109/L), along with bone marrow dysplasia. Clonal cytogenetic abnormalities occur in ∼20-30% of patients, while >90% have gene mutations. Mutations involving TET2 (∼60%), SRSF2 (∼50%), ASXL1 (∼40%), and RAS (∼30%) are frequent; with only ASXL1 mutations negatively impacting overall survival. Two molecularly integrated, CMML-specific prognostic models include; the Groupe Français des Myélodysplasies (GFM) and the Molecular Mayo Model (MMM). The GFM model segregates patients into 3 groups based on: age >65years, WBC >15×109/L, anemia, platelets 9/L, and ASXL1 mutation status, with respective median survivals of 56 (low), 27.4 (intermediate), and 9.2 (high) months. The MMM is based on ASXL1 mutational status, absolute monocyte count >10×109/L, hemoglobin

Original languageEnglish (US)
Pages (from-to)631-642
Number of pages12
JournalAmerican Journal of Hematology
Volume91
Issue number6
DOIs
StatePublished - Jun 1 2016

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Leukemia, Myelomonocytic, Chronic
Risk Management
Mutation
Molecular Models
Developmental Bone Disease
Survival
Myelodysplastic Syndromes
Hematopoietic Stem Cells
Chromosome Aberrations
Anemia
Monocytes
Hemoglobins
Blood Platelets
Bone Marrow
Genes
Neoplasms

ASJC Scopus subject areas

  • Hematology

Cite this

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title = "Chronic myelomonocytic leukemia: 2016 update on diagnosis, risk stratification, and management",
abstract = "Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic stem cell disorder characterized by overlapping features of myelodysplastic syndromes and myeloproliferative neoplasms. Diagnosis is based on the presence of persistent (>3months) peripheral blood monocytosis (>1×109/L), along with bone marrow dysplasia. Clonal cytogenetic abnormalities occur in ∼20-30{\%} of patients, while >90{\%} have gene mutations. Mutations involving TET2 (∼60{\%}), SRSF2 (∼50{\%}), ASXL1 (∼40{\%}), and RAS (∼30{\%}) are frequent; with only ASXL1 mutations negatively impacting overall survival. Two molecularly integrated, CMML-specific prognostic models include; the Groupe Fran{\cc}ais des My{\'e}lodysplasies (GFM) and the Molecular Mayo Model (MMM). The GFM model segregates patients into 3 groups based on: age >65years, WBC >15×109/L, anemia, platelets 9/L, and ASXL1 mutation status, with respective median survivals of 56 (low), 27.4 (intermediate), and 9.2 (high) months. The MMM is based on ASXL1 mutational status, absolute monocyte count >10×109/L, hemoglobin",
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N2 - Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic stem cell disorder characterized by overlapping features of myelodysplastic syndromes and myeloproliferative neoplasms. Diagnosis is based on the presence of persistent (>3months) peripheral blood monocytosis (>1×109/L), along with bone marrow dysplasia. Clonal cytogenetic abnormalities occur in ∼20-30% of patients, while >90% have gene mutations. Mutations involving TET2 (∼60%), SRSF2 (∼50%), ASXL1 (∼40%), and RAS (∼30%) are frequent; with only ASXL1 mutations negatively impacting overall survival. Two molecularly integrated, CMML-specific prognostic models include; the Groupe Français des Myélodysplasies (GFM) and the Molecular Mayo Model (MMM). The GFM model segregates patients into 3 groups based on: age >65years, WBC >15×109/L, anemia, platelets 9/L, and ASXL1 mutation status, with respective median survivals of 56 (low), 27.4 (intermediate), and 9.2 (high) months. The MMM is based on ASXL1 mutational status, absolute monocyte count >10×109/L, hemoglobin

AB - Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic stem cell disorder characterized by overlapping features of myelodysplastic syndromes and myeloproliferative neoplasms. Diagnosis is based on the presence of persistent (>3months) peripheral blood monocytosis (>1×109/L), along with bone marrow dysplasia. Clonal cytogenetic abnormalities occur in ∼20-30% of patients, while >90% have gene mutations. Mutations involving TET2 (∼60%), SRSF2 (∼50%), ASXL1 (∼40%), and RAS (∼30%) are frequent; with only ASXL1 mutations negatively impacting overall survival. Two molecularly integrated, CMML-specific prognostic models include; the Groupe Français des Myélodysplasies (GFM) and the Molecular Mayo Model (MMM). The GFM model segregates patients into 3 groups based on: age >65years, WBC >15×109/L, anemia, platelets 9/L, and ASXL1 mutation status, with respective median survivals of 56 (low), 27.4 (intermediate), and 9.2 (high) months. The MMM is based on ASXL1 mutational status, absolute monocyte count >10×109/L, hemoglobin

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