Myelodysplastic syndrome(MDS)
Myelodysplastic syndrome (MDS) consists of a group of clonal haematopoietic disorders which represent steps in the progression to the development of leukaemia.MDS presents with consequences of bone marrow failure (anaemia, recurrent infections or bleeding),usually in older people (median age at diagnosis is 69 years)
The overall incidence is 4/100 000 in the population, rising to more than 30/100 000 in the over-seventies
The blood film is characterised by cytopenias and abnormal-looking (dysplastic) blood cells, including macrocytic red cells and hypogranular neutrophils with nuclear hyper-or hyposegmentation.
The bone marrow is hypercellular, with dysplastic changes in all three cell lines. Blast cells may be increased but do not reach the 20% level that indicates acute leukaemia
WHO classification of myelodysplasticsyndromes (MDSs
Revised International Prognostic ScoringSystem and outcomes in myelodysplasia*
Management
*For the vast majority of patients who are elderly, the disease is incurable, and supportive care with red cell and platelet transfusions is the mainstay of treatment*For younger patients with higher-risk disease, allogeneic HSCT may afford a cure.
*del(5q) responds well to the immunomodulatory drug lenalidomide, with two-thirds of anaemic patients becoming transfusion-independent for up to 2 years
*A trial of erythropoietin and granulocyte–colony-stimulating factor (G–CSF) is recommended in some patients with early disease to improve haemoglobin and white cell counts.
*Hypomethylating agent azacytidine has improved survival by a median of 9 months for high-risk patients, is recommended for those not eligible for transplantation