Importance of Genetics in the Clinical Management of Chronic Myelomonocytic Leukemia
Menée sur 312 patients atteints d'une leucémie myélomonocytaire chronique, puis validée sur une cohorte indépendante comportant 165 autres patients, cette étude évalue, du point de vue de la survie globale, la valeur pronostique d'un nouveau système de score basé sur le statut mutationnel du gène ASXL1, l'âge et des données cliniques (numération plaquettaire, numération des globules blancs, taux d'hémoglobine)
Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic malignancy that is characterized by dysplasia, peripheral monocytosis, ineffective hematopoiesis, and an increased risk of transformation to acute myeloidleukemia. Because the clinical phenotype of CMML displays features of both myelodysplastic syndromes (MDSs) and myeloproliferative neoplasms (MPNs), the classification of CMML has historically been a dynamic process. Before 2001, CMML was classified as a subtype of MDSs, as initially reported by the French-American-British Group, because of its characteristic dysplasia, cytopenias, and acute myeloid leukemia transformation rate. However, as it became increasingly clear that CMML is clinically distinct from MDS, CMML was placed in a provisional category within the World Health Organization (WHO classification of hematologic neoplasms known as MDS/MPN overlap diseases. This classification schema was formalized by WHO in 2008 and defines CMML as having a persistent peripheral blood monocytosis greater than 1X10
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/L, dysplasia in at least one hematopoietic lineage, and less than 20% myeloblasts and promonocytes in the peripheral blood and bone marrow
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. Although it is clinically heterogeneous, the overall survival in CMML is poor. It is the most aggressive chronic myeloid malignancy, with 3-year survival on the order of 20%...
Journal of Clinical Oncology , éditorial en libre accès, 2013