Acute Chest Syndrome in Children with Sickle Cell Disease: Case Report and Review of the Literature

Abstract

Sickle cell anemia (SCA) is the most common hemoglobinopathy all over the world. While the overall incidence of sickle cell disease trait in Turkey is %0.3-0.6, especially some places in Çukurova area it reaches the level of 3-44%. Painful vaso- occlusive crisis which is the most important complication of this disease, is predicted to increase in frequency and HbS polymerization is responsible in its pathophysiology. As an acute complication of SCA, acute chest syndrome is the second most common cause of hospitalization and mortality. İnitial approach to patients with an acute chest syndrome includes supportive treatments. Whereas, solely transfusion is not enough for treatment if there is worsening in respiratory signs and chest radiography; deepening of hypoxemia which requires an oxygen support. Therapeutic red cell apheresis can be used with the aim to treat acute complication of sickle cell disease. In this article we present successful therapeutic red cell apheresis and supportive treatments that we practise in our case who developed acute chest syndrome, and followed up by us with the diagnosis of sickle cell disease.