TY - JOUR
T1 - Chronic Myelomonocytic Leukemia
T2 - Insights into Biology, Prognostic Factors, and Treatment
AU - Coltro, Giacomo
AU - Patnaik, Mrinal M.
N1 - Publisher Copyright:
© 2019, Springer Science+Business Media, LLC, part of Springer Nature.
PY - 2019/11/1
Y1 - 2019/11/1
N2 - Purpose of Review: Chronic myelomonocytic leukemia (CMML) is a clonal hematological malignancy characterized by both dysplastic and proliferative features, with an inherent risk for leukemic transformation. With the help of this review, we aim to summarize key concepts with regards to CMML biology, diagnosis, risk stratification, and therapeutics. Recent Findings: Based on recent studies, CMML is hallmarked by a relatively low genetic complexity, which contrasts with a compelling phenotypical heterogeneity, largely driven by epigenetic mechanisms. Recent advances in the characterization of CMML biology has led to an improvement in risk-stratification, by means of incorporating prognostically relevant gene mutations. This, however, has not significantly impacted available therapies and outcomes continue to remain poor. Summary: Advances in CMML biology have better explained the phenotypic heterogeneity, while continuing to define the genetic and epigenetic landscape. In spite of recent advances, limited effective therapies exist and developing rationally derived therapeutic approaches is much needed.
AB - Purpose of Review: Chronic myelomonocytic leukemia (CMML) is a clonal hematological malignancy characterized by both dysplastic and proliferative features, with an inherent risk for leukemic transformation. With the help of this review, we aim to summarize key concepts with regards to CMML biology, diagnosis, risk stratification, and therapeutics. Recent Findings: Based on recent studies, CMML is hallmarked by a relatively low genetic complexity, which contrasts with a compelling phenotypical heterogeneity, largely driven by epigenetic mechanisms. Recent advances in the characterization of CMML biology has led to an improvement in risk-stratification, by means of incorporating prognostically relevant gene mutations. This, however, has not significantly impacted available therapies and outcomes continue to remain poor. Summary: Advances in CMML biology have better explained the phenotypic heterogeneity, while continuing to define the genetic and epigenetic landscape. In spite of recent advances, limited effective therapies exist and developing rationally derived therapeutic approaches is much needed.
KW - Chronic myelomonocytic leukemia
KW - Clonal architecture
KW - Myelodysplastic syndromes
KW - Myeloproliferative neoplasms
KW - Prognostication
KW - Target therapy
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U2 - 10.1007/s11912-019-0855-6
DO - 10.1007/s11912-019-0855-6
M3 - Review article
C2 - 31728739
AN - SCOPUS:85075058812
SN - 1523-3790
VL - 21
JO - Current oncology reports
JF - Current oncology reports
IS - 11
M1 - 101
ER -