McLaughlin JF, Ballas SK. High mortality among children with sickle cell anemia and overt stroke who discontinue blood transfusion after transition to an adult program. Transfusion. 2016;56:1014-1021.

Simple and exchange blood transfusions are standard care for prevention of recurrent strokes in patients with sickle cell anemia.  Blood transfusions reduce the number of sickle cells in circulation and thus improve oxygenation and blood rheology. Problems arise, however, when children transition to adult care; not all adults are compliant with treatment.  Researchers followed 22 sickle cell patients between the age of 18 and 28 that had a history of overt strokes while transitioning to adult care.  Non-compliance and refusal of blood transfusions occurred among 8 patients, and was associated with death within 1-5 years from stroke or multi-organ failure.  The 14 compliant patients were rarely hospitalized for serious conditions, although some did experience alloimmunization (7/14) and iron overload (11/14) complications from blood transfusions.  This study is important in pointing out the clinicians to be aware of highly encouraging young adults with sickle cell anemia to continue chronic (simple or exchange) blood transfusions when transitioning to adult care.

This summary is prepared by İbrahim Eker,M.D.

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