TY - JOUR
T1 - Current management of patients with chronic myelomonocytic leukemia
AU - Alfonso, Ana
AU - Montalban-Bravo, Guillermo
AU - Garcia-Manero, Guillermo
N1 - Publisher Copyright:
© 2016 Wolters Kluwer Health, Inc. All rights reserved.
PY - 2017
Y1 - 2017
N2 - Purpose of review The present review will focus on the current management of patients with chronic myelomonocytic leukemia (CMML) as well as in future therapeutic perspectives. Recent findings CMML is a clonal hematopoietic stem cell disorder characterized by peripheral blood monocytosis and myelodysplastic and myeloproliferative alterations in the bone marrow. Clinical behavior of the disease can be heterogeneous, with some patients having an indolent form of the disease, whereas others experience an aggressive course with decreased survival and eventual transformation to leukemia. Multiple studies have helped define the clinical, cytogenetic and mutational prognostic features of the disease. In addition, several prognostic scoring systems have been developed for patients with CMML. Incorporation of mutation data, particularly presence of frameshift and nonsense ASXL1 mutations, into these models seems to be allowing to further improve our ability to predict patient outcomes. Summary Prognosis of patients with CMML is heterogeneous. Incorporation of mutational data into current clinical prognostic models has allowed to improve our ability to predict patient outcomes. Allogeneic stem cell transplantation remains the only potentially curative treatment for patients with CMML but is only an option for a subset of patients. For this reason, hypomethylating agents such as 5-azacitidine and decitabine have become the backbone of current therapy for patients with CMML, but new therapeutic strategies are required to improve their outcomes.
AB - Purpose of review The present review will focus on the current management of patients with chronic myelomonocytic leukemia (CMML) as well as in future therapeutic perspectives. Recent findings CMML is a clonal hematopoietic stem cell disorder characterized by peripheral blood monocytosis and myelodysplastic and myeloproliferative alterations in the bone marrow. Clinical behavior of the disease can be heterogeneous, with some patients having an indolent form of the disease, whereas others experience an aggressive course with decreased survival and eventual transformation to leukemia. Multiple studies have helped define the clinical, cytogenetic and mutational prognostic features of the disease. In addition, several prognostic scoring systems have been developed for patients with CMML. Incorporation of mutation data, particularly presence of frameshift and nonsense ASXL1 mutations, into these models seems to be allowing to further improve our ability to predict patient outcomes. Summary Prognosis of patients with CMML is heterogeneous. Incorporation of mutational data into current clinical prognostic models has allowed to improve our ability to predict patient outcomes. Allogeneic stem cell transplantation remains the only potentially curative treatment for patients with CMML but is only an option for a subset of patients. For this reason, hypomethylating agents such as 5-azacitidine and decitabine have become the backbone of current therapy for patients with CMML, but new therapeutic strategies are required to improve their outcomes.
KW - Chronic myelomonocytic leukemia
KW - decitabine
KW - management
KW - mutations
KW - prognosis
KW - treatment
UR - http://www.scopus.com/inward/record.url?scp=84995511361&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84995511361&partnerID=8YFLogxK
U2 - 10.1097/CCO.0000000000000337
DO - 10.1097/CCO.0000000000000337
M3 - Review article
C2 - 27849645
AN - SCOPUS:84995511361
SN - 1040-8746
VL - 29
SP - 79
EP - 87
JO - Current opinion in oncology
JF - Current opinion in oncology
IS - 1
ER -