Chronic myelomonocytic leukaemia: a concise clinical and pathophysiological review.
Chronic myelomonocytic leukaemia (CMML) is a clonal haematopoietic stem cell disorder with myelodysplastic and myeloproliferative overlap features, and an inherent tendency to transform to acute myeloid leukaemia. Approximately 30% of patients present with clonal cytogenetic abnormalities, while almost 90% have molecular aberrations involving epigenetic regulation, the spliceosome component machinery, tumour suppressor genes and transcription factors/regulators. Numerous prognostic models exist for CMML, with more recent models incorporating prognostic mutations, such as those involving ASXL1. Other variables that seem to consistently affect outcomes include the degree of leucocytosis/monocytosis, anaemia and thrombocytopenia. Allogeneic stem cell transplant remains the only curative option for CMML, while hypomethylating agents can be used for transplant-ineligible patients or those without suitable stem cell sources. Targeting biological pathways activated in CMML offers potential hope for more effective and less toxic therapies.
Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA.
Mrinal M
MM
Patnaik
Sameer A
SA
Parikh
Curtis A
CA
Hanson
Ayalew
A
Tefferi
D006801:Humans; D015477:Leukemia, Myelomonocytic, Chronic / Q000235:genetics / Q000473:pathology / Q000503:physiopathology* / Q000628:therapy*; D011379:Prognosis; D033581:Stem Cell Transplantation / Q000379:methods; D014184:Transplantation, Homologous
ASXL1; allogeneic stem cell transplant; chronic myelomonocytic leukaemia; hypomethylating agents; prognosis