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Pathogenesis of myelodysplastic syndromes: an overview of molecular and non-molecular aspects of the disease
- Source :
- Blood research
- Publication Year :
- 2014
-
Abstract
- Myelodysplastic syndromes (MDS) are a group of clonal disorders arising from hematopoietic stem cells generally characterized by inefficient hematopoiesis, dysplasia in one or more myeloid cell lineages, and variable degrees of cytopenias. Most MDS patients are diagnosed in their late 60s to early 70s. The estimated incidence of MDS in the United States and in Europe are 4.3 and 1.8 per 100,000 individuals per year, respectively with lower rates reported in some Asian countries and less well estimated in other parts of the world. Evolution to acute myeloid leukemia can occur in 10-15% of MDS patients. Three drugs are currently approved for the treatment of patients with MDS: immunomodulatory agents (lenalidomide), and hypomethylating therapy [HMT (decitabine and 5-azacytidine)]. All patients will eventually lose their response to therapy, and the survival outcome of MDS patients is poor (median survival of 4.5 months) especially for patients who fail (refractory/relapsed) HMT. The only potential curative treatment for MDS is hematopoietic cell transplantation. Genomic/chromosomal instability and various mechanisms contribute to the pathogenesis and prognosis of the disease. High throughput genetic technologies like single nucleotide polymorphism array analysis and next generation sequencing technologies have uncovered novel genetic alterations and increased our knowledge of MDS pathogenesis. We will review various genetic and non-genetic causes that are involved in the pathogenesis of MDS.
- Subjects :
- Oncology
medicine.medical_specialty
Myeloid
business.industry
Myelodysplastic syndromes
Decitabine
Myeloid leukemia
Hematology
Disease
Review Article
Pathogenesis
medicine.disease
Transplantation
medicine.anatomical_structure
Dysplasia
Internal medicine
hemic and lymphatic diseases
Immunology
medicine
MDS
Molecular mutation
business
medicine.drug
Lenalidomide
Subjects
Details
- ISSN :
- 2287979X
- Volume :
- 49
- Issue :
- 4
- Database :
- OpenAIRE
- Journal :
- Blood research
- Accession number :
- edsair.doi.dedup.....d4a6424e8d070dd0c4a98e45938241bf