Myelodysplastic syndromes

L Adès, R Itzykson, P Fenaux - The Lancet, 2014 - thelancet.com
L Adès, R Itzykson, P Fenaux
The Lancet, 2014thelancet.com
Myelodysplastic syndromes are clonal marrow stem-cell disorders, characterised by
ineffective haemopoiesis leading to blood cytopenias, and by progression to acute myeloid
leukaemia in a third of patients. 15% of cases occur after chemotherapy or radiotherapy for a
previous cancer; the syndromes are most common in elderly people. The pathophysiology
involves cytogenetic changes with or without gene mutations and widespread gene
hypermethylation at advanced stages. Clinical manifestations result from cytopenias …
Summary
Myelodysplastic syndromes are clonal marrow stem-cell disorders, characterised by ineffective haemopoiesis leading to blood cytopenias, and by progression to acute myeloid leukaemia in a third of patients. 15% of cases occur after chemotherapy or radiotherapy for a previous cancer; the syndromes are most common in elderly people. The pathophysiology involves cytogenetic changes with or without gene mutations and widespread gene hypermethylation at advanced stages. Clinical manifestations result from cytopenias (anaemia, infection, and bleeding). Diagnosis is based on examination of blood and bone marrow showing blood cytopenias and hypercellular marrow with dysplasia, with or without excess of blasts. Prognosis depends largely on the marrow blast percentage, number and extent of cytopenias, and cytogenetic abnormalities. Treatment of patients with lower-risk myelodysplastic syndromes, especially for anaemia, includes growth factors, lenalidomide, and transfusions. Treatment of higher-risk patients is with hypomethylating agents and, whenever possible, allogeneic stem-cell transplantation.
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