EFFECT OF TRANSFUSION IN ACUTE CHEST SYNDROME OF SICKLE-CELL DISEASE

Objective: To study the effects of transfusion on the clinical course and oxygenation indexes of children with sickle cell disease and acute chest syndrome. Methods: During a 2-year period, 36 children with sickle cell disease admitted with a total of 40 episodes of acute chest syndrome were examined, Patients were given a clinical severity score indicative of the degree of respiratory distress. Arterial blood gas values were determined 4 to 24 hours before and 12 to 24 hours after transfusion, and indexes of oxygenation were calculated; six patients who were not given transfusions also had blood gases measured on admission and approximately 24 hours later for comparison. Results: Blood transfusion was administered during 27 episodes (67.5%); 20 children received a simple packed cell transfusion, four had a partial packed cell exchange transfusion, and three had a simple transfusion followed by whole blood exchange transfusion because of worsening clinical symptoms. Although there was no significant change in oxygenation indexes for the six patients not treated with transfusion, there was significant improvement in all indexes after transfusion. The transfused group had more severe disease on admission, but there was no significant difference in duration of fever, tachypnea, retractions, or hospital stay between the transfusion and the nontransfusion groups. Conclusion: Blood transfusion, even simple transfusion of packed erythrocytes, significantly improves oxygenation in children with acute chest syndrome and is a valuable adjunct to therapy.