Chronic myelomonocytic leukemia: 2013 update on diagnosis, risk stratification, and management

Disease overviewChronic 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. DiagnosisThe 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. Risk stratificationSeveral 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. Risk‐adapted therapyThe Food and Drug Administration has appr...
Source: American Journal of Hematology - Category: Hematology Authors: Tags: Annual Clinical Updates in Hematological Malignancies: A Continuing Medical Education Series Source Type: research