Περίληψη:
Introduction
Chronic myelomonocytic leukemia (CMML) is a clonal myeloid neoplasm,
denoted by overlapping myelodysplastic and myeloproliferative features,
with poor overall survival and high transformation rate to acute myeloid
leukemia.
Areas covered
This review, following a thorough Medline search of pertinent published
literature, discusses the diagnostic criteria, the pathogenesis, and the
complex genetic landscape of the disease. Prognostication, response
criteria, therapeutic management of patients, efficacy of established
and novel treatment modalities are thoroughly reviewed.
Expert opinion
Cytogenetic abnormalities and mutations in genes involved in epigenetic
and transcriptional regulation, and cell-signaling are abundant in CMML
and implicated in its complex pathogenesis. As presence of these
mutations carry a prognostic impact, they are increasingly incorporated
in risk-stratification schemes. Novel response criteria have been
proposed, considering the unique features of the disease. Although
allogeneic hematopoietic stem cell transplantation remains the only
treatment with curative intent, it is reserved for a minority of
patients; therefore, there is an unmet need for optimizing treatment
modalities, such as hypomethylating agents, and introducing novel
agents, which could substantially improve survival and quality of life
of CMML patients. Clinical trials dedicated specifically to CMML are
needed to explore the efficacy and safety of novel treatment modalities.
Συγγραφείς:
Thomopoulos, Thomas P.
Bouhla, Anthi
Papageorgiou, Sotirios G.
and Pappa, Vasiliki
