Onkológia 4/2014
Treatment of myelodysplastic syndrome with high-risk groups
Background: Myelodysplastic syndrome (MDS) is malignant hematopoetic stem-cell disease characterized by ineffective hematopoiesis. MDS is diagnosed by cytology, with consideration of the degree of dysplasia and the percentage of blast cells in the blood and bone marrow, and on a cytogenetic basis. Chromosomal analysis is necessary for prognostication. The Revised International Prognosis Scoring System (IPSS-R) enables more accurate prediction of the course of disease and choice of appropriate therapeutic strategy by dividing patients into a number of low- and high-risk groups. Methods: We selectively searched the PubMed database for pertinent articles and guidelines from the years 2000-2014. We used the search term “myelodysplastic syndrome”. Results: The median survival time ranges from a few months (0,4 year) to many years (8,8 years). Prognosis of the high-risk groups patients (intermediate -2, high, very high risk) is not good with the median survival time ranges from 0,4 to 1,2 years with high rate of transformation to acute myeloid leukemia. The approved treatments for patients in high-risk groups is 5-azacytidine and patients up to age 70 who have no major accompanying illnesses should be offered allogenic stem-cell transplantation (SCT) with curative intent. The cure rates range from 35% to 40% (disease free survival DFS). The most complications of allogenic SCT are infections, mucositis, hemorrhages and graft-versus-host diseases (GVHD). The benefits of HSCT need to be balanced against risks of nonrelapse mortality (NRM), GVHD and immune dysfunction. Relaps remains a leading cause for treatment failure after hematopoetic SCT in patients in high-risk groups. Conclusion: Myelodysplastic syndrome is treated on an individualized, risk-adapted basis after precise diagnostic evaluation and after assessment of the prognosis. More studies are needed so that stage-adapted treatment can be improved still further.
