title: Chronic myelomonocytic leukemia: 2013 update on diagnosis, risk stratification, and management.
abstract: BACKGROUND
  Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic stem cell disorder that is classified as a myelodysplastic/myeloproliferative neoplasm by the 2008 World Health Organization classification of hematopoietic tumors. It is characterized by absolute monocytosis (>1 × 10⁹/L) in the peripheral blood that persists for at least 3 months.
  
  
  METHODS
  The diagnosis of CMML rests on a combination of morphologic, histopathologic and chromosomal abnormalities in the bone marrow. It is important to exclude other myeloproliferative neoplasms and infectious/autoimmune conditions that can cause monocytosis.
  
  
  METHODS
  Several CMML-specific prognostic models incorporating novel mutations have been recently reported. The Mayo prognostic model classified CMML patients into three risk groups based on: increased absolute monocyte count, presence of circulating blasts, hemoglobin <10 gm/dL and platelets <100 × 10⁹/L. The median survival was 32 months, 18.5 months and 10 months in the low, intermediate, and high-risk groups, respectively. The Groupe Francophone des (GFM) score segregated CMML patients into three risk groups based on: age >65 years, WBC >15 × 10⁹/L, anemia, platelets <100 × 10⁹/L, and ASXL1 mutation status. After a median follow-up of 2.5 years, survival ranged from not reached in the low-risk group to 14.4 months in the high-risk group.
  
  
  METHODS
  The Food and Drug Administration has approved azacitidine and decitabine for the treatment of patients with CMML. An allogeneic stem cell transplant can potentially offer a curative option to a subset of CMML patients. It is hoped that with the discovery of several novel mutations, targeted therapies will become available in the near future.
authors: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors: Sameer A
authors: SA
authors: Parikh
authors: Ayalew
authors: A
authors: Tefferi
mesh_terms: D000964:Antimetabolites, Antineoplastic / Q000009:adverse effects / Q000627:therapeutic use; D001374:Azacitidine / Q000009:adverse effects / Q000031:analogs & derivatives / Q000627:therapeutic use; D019175:DNA Methylation / Q000187:drug effects; D003663:Decision Trees; D000077209:Decitabine; D003937:Diagnosis, Differential; D006801:Humans; D015477:Leukemia, Myelomonocytic, Chronic / Q000175:diagnosis* / Q000188:drug therapy* / Q000235:genetics / Q000503:physiopathology; D007964:Leukocytosis / Q000209:etiology; D009000:Monocytes / Q000276:immunology; D009154:Mutation; D017410:Practice Guidelines as Topic; D011379:Prognosis
chemical_list: D000964:Antimetabolites, Antineoplastic; D000077209:Decitabine; D001374:Azacitidine