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2 studies provide insight into stroke risk and prevention in young sickle cell anemia patients
Date:12/6/2010

Monthly blood transfusions combined with daily medication to remove the resulting excess iron remains the best approach for reducing the risk of recurrent strokes in young patients with sickle cell anemia, according to a preliminary analysis of a multicenter trial that includes St. Jude Children's Research Hospital.

The study compared the efficacy of two treatments for the potentially life-threatening problem of iron overload caused by chronic transfusion therapy. The transfusions are used to guard against additional strokes in young sickle cell anemia patients. The trial, known as SWiTCH or Stroke with Transfusion Changing to Hydroxyurea, was halted in May after an interim safety review determined the alternative therapy was not significantly better than the standard treatment at reducing iron buildup and was associated with an increased stroke risk.

Russell Ware, M.D., Ph.D., chair of the St. Jude Children's Research Hospital Department of Hematology and principal investigator of SWiTCH, discussed the results at the 52nd Annual Meeting of the American Society of Hematology. The meeting is being held December 4 -7 in Orlando, Fla.

Jonathan Flanagan, Ph.D., a staff scientist at St. Jude, presented results of another study that provide the first independent validation of an association between five common genetic variations and stroke risk in young sickle cell patients.

Between 70,000 and 100,000 individuals in the U.S. have sickle cell anemia. They make an abnormal hemoglobin molecule that sometimes takes on a twisted or sickle shape, disrupting blood flow and oxygen delivery throughout the body. Patients are vulnerable to a variety of problems, including organ damage, episodes of acute pain and stroke. Five to 10 percent of patients will suffer strokes before their 20th birthday. Up to 90 percent of patients will experience a recurrence.

Flanagan and his colleagues tried to validate earlier reports linking 38 genet
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Contact: Summer Freeman
summer.freeman@stjude.org
901-595-3061
St. Jude Children's Research Hospital
Source:Eurekalert

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