Hippokratia 2007; 11 (4): 178-182
M. Diamantidis, S. Dimoudis, Ph. Klonizakis, K. Badekas, K. Koutourli, S. Haralambidou-Vranitsa, E. Ioannidou-Papagiannaki
The myelodysplastic syndromes (MDS) remain challenging to the clinician in terms of diagnosis and management. The diagnosis is essentially one of exclusion in first ruling out other disorders that can also cause peripheral blood/bone marrow cell dysplasia and cytopenias. Recent studies implicate extensive apoptosis as the explanation of the paradoxical observation of marrow hyperplasia, but peripheral blood cytopenia. The clonal nature of MDS places it also at continual risk for transformation to acute leukemia. Predicting overall survival as well as the risk of acute myeloid leukaemia (AML) transformation has been improved by the development of the International Prognostic Scoring System (IPSS).
Management of MDS can now be based on the patient???s respective prognostic subgrouping. Low-risk patients should be considered for hematopoietic growth factor singly or in combination, while high-risk patients should be offered AMLinduction therapy or novel therapeutic agents. Common complications are neutropenias with recurrent infections and red cell transfusion dependence. Future advances upon understanding the molecular details of the MDS clone should ultimately improve the care of patients with MDS.
Keywords: myelodysplastic syndromes, apoptosis, prognostic system IPSS, acute myeloid leukaemia, therapeutic strategies