گزارش يك مورد ايسكمي ميوكارد در بيمار مبتلا به سيكل βº تالاسمي و پاسخ آن به درمان با هيدروكسي اوره

Myocardial ischemia in a patient with sickle cell βº thalassemia and its response to hydroxyurea: a case report

Sickle cell Patients with either homozygous or heterozygous forms of βº -sickle cell thalassemia are at risk of developing cardiac problems, including the occurrence of sudden unexpected death. Sudden death can arise from significant morphological abnormalities in the cardiac conduction system. However, myocardial infarction is a rare occurrence in young adults with sickle cell disease and may be attributable, in most cases, to acute micro-vascular occlusion of small vessels. In this report, we present an Abadanian, 22 year-old young adult man every Arab ethnicity, whoʹs disease started six years ago with bone pains, and was under six-monthly blood transfusion for control of anemia. Recently he was admitted for bone and chest pain. Perfusion thaliam-scan suggested a myocardial ischemia for which he was treated with hydroxylurea. In view of the fact that the hallmark of sickle cell disease is micro-vascular occlusion, treatment with hydroxyurea not only improved bone and chest pain but also, in this case, caused the disappearance of his myocardial ischemia, raising in Hb-F level and reduced the rate of blood transfusion.