Myelodysplastic Syndromes

Published on 04/03/2015 by admin

Filed under Hematology, Oncology and Palliative Medicine

Last modified 04/03/2015

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Chapter 99

Myelodysplastic Syndromes

Summary of Key Points

Epidemiology

• The most important risk factor for MDS is aging. The risk of developing MDS increases greatly after age 65 years. The median age at diagnosis in the United States is approximately 71 years.

• Approximately 10% to 15% of MDS cases arise as a consequence of therapeutic or environmental exposure to a DNA damaging agent; these are termed secondary or therapy-related MDS. In patients who had been exposed to ionizing radiation or alkylating agents, abnormalities of chromosomes 5 and 7 are common. Patients treated with topoisomerase II inhibitors may develop rearrangements of the MLL gene at 11q23 or the MECOM (MDS1/EVI1) locus on chromosome 3q21q26.

• Patients who develop MDS before age 40 years without a recognized toxin exposure may have a germline DNA repair defect or congenital marrow failure syndrome, such as Fanconi anemia or dyskeratosis congenita. Chromosome breakage analysis and telomere length analysis is appropriate in such patients.