What is Myelodysplasia

The myelodysplastic syndromes (MDS, formerly known as "preleukemia") are a diverse collection of haematological conditions united by ineffective production of blood cells and varyi...

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Myelodysplasia Information

The myelodysplastic syndromes (MDS, formerly known as "preleukemia") are a diverse collection of haematological conditions united by ineffective production of blood cells and varying risks of transformation to acute myelogenous leukemia. Anemia requiring chronic blood transfusion is frequently present. Although not a true malignant neoplasm, MDS is nevertheless classified within the hematological neoplasms.

The average age at diagnosis for MDS is about 65 years, but pediatric cases have been reported. Some patients have a history of exposure to chemotherapy (especially alkylating agents such as melphalan, mustard, cyclophosphamide, busulfan, and chlorambucil) or radiation (therapeutic or accidental), or both (e.g., at the time of stem cell transplantation for another disease). Workers in some industries with heavy exposure to hydrocarbons such as the petroleum industry have a slightly higher risk of contracting the disease than the general population. Males are slightly more frequently affected than females. Xylene and benzene exposure has been associated with myelodysplasia.

The goals of therapy are to control symptoms, improve quality of life, improve overall survival, and decrease progression to acute myelogenous leukemia.

The IPSS scoring system can help triage patients for more aggressive treatment (i.e. bone marrow transplant) as well as help determine the best timing of this therapy. Supportive care with blood product support and hematopoeitic growth factors (e.g. erythropoietin) is the mainstay of therapy. Chemotherapy with 5-azacytidine has been shown to decrease blood transfusion requirements and the progression to AML. Lenalidomide is the newest addition in treatment options and was approved by the FDA in December 2005. It has the best activity in patients with the 5q- cytogenetic abnormality, with or without additional abnormalities.

Bone marrow transplant, particularly in younger, more severely affected patients, offers the potential for curative therapy. Success of bone marrow transplantation has been found to correlate with severity of MDS as determined by the IPSS score.

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