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Chronic myelomonocytic leukaemia: a concise clinical and pathophysiological review
Author(s) -
Patnaik Mrinal M.,
Parikh Sameer A.,
Hanson Curtis A.,
Tefferi Ayalew
Publication year - 2014
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.12756
Subject(s) - monocytosis , epigenetics , stem cell , chronic myelomonocytic leukemia , medicine , haematopoiesis , decitabine , myelodysplastic syndromes , cancer research , myeloid , hematology , oncology , myeloid leukaemia , somatic evolution in cancer , immunology , dna methylation , biology , bone marrow , gene , genetics , cancer , gene expression
Summary 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 ASXL 1 . 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.