Disease Information
Leukemias / Lymphomas: Myelodysplasia (MDS)
Alternate Names: MDS
Definition
Myelodysplasia (MDS) refers to a group of disorders in which the bone marrow does not function normally and produces insufficient number of normal blood cells.
MDS affects the production of any, and occasionally all, types of blood cells including red blood cells, platelets, and white blood cells (cytopenias).
About 50 percent of pediatric myelodysplasia can be classified in five types of MDS: refractory anemia, refractory anemia with ring sideroblasts, refractory anemia with excess blasts, refractory anemia with excess blasts in transformation, and chronic myelomonocytic leukemia.
The remaining 50 percent typically present with isolated or combined cytopenias such as anemia, leucopenia and/or thrombocytopenia (low platelet count). Although chronic, MDS progresses to become acute myeloid leukemia (AML) in about 30 percent of patients.
Incidence
- Fewer than 100 new cases of myelodysplasia are reported in the United States each year in children.
- MDS is found most often in adults over the age of 50 (median age 65 years old), but is also seen in children of all ages.
Influencing Factors
- There is a slightly higher incidence of myelodysplasia in males than in females.
- Genetic and environmental factors have been implicated in the etiology of MDS. Exposure to excessive radiation, some toxic chemicals, certain medicines, and viral infections may cause MDS in some people.
Survival Rates
Up to 60 percent of patients that receive bone marrow transfusions achieve long-term survival.
Treatment Strategies
- Allogeneic hematopoietic stem cell transplantation (transfusions using marrow, peripheral blood or cord blood from a compatible donor) is the most effective form of treatment for most types of myelodysplasia.
- Chemotherapy is a form of treatment for some patients with a high risk of MDS progressing into AML.
- Supportive treatments include blood and platelet transfusions and antibiotics.
Current Research
Most St. Jude myelodysplasia patients are enrolled on our front-line AML or hematopoietic stem cell transplantation protocols.
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