Comparison of Automated Red Cell Exchange Transfusion and Simple Transfusion for the Treatment of Children With Sickle Cell Disease Acute Chest Syndrome
ABSTRACT Both simple transfusion (ST) of packed red blood cells and automated red cell exchange (RCE) are used in the treatment of acute chest syndrome (ACS). We report our experience using each of these modalities for the treatment of ACS.
Retrospective chart review of patients with ACS treated with ST only (51 episodes, ST group) or RCE performed either at diagnosis (U-RCE group, 15 episodes) or after ST (ST + RCE group, 15 episodes).
The mean clinical respiratory score (CRS) at diagnosis was significantly higher in the U-RCE group than in the ST group, but there were no significant differences among the other groups. The CRS and WBC each decreased significantly after simple transfusion in the ST group and after RCE in the U-RCE group, but both the CRS and WBC increased significantly, and the mean platelet count fell significantly, after simple transfusion in the ST + RCE group. Only patients in the ST + RCE group required mechanical ventilation. There were no significant differences in length of stay (LOS) or total hospital charges among any of the groups, probably due to the small sample size.
We conclude that the CRS identifies the patients who are most severely affected with ACS, and that upfront RCE is a safe and effective treatment for these patients. Additional work is needed to develop a method to predict which of the apparently less severely affected patients will fail to improve after simple transfusion and should receive upfront RCE. Pediatr Blood Cancer © 2013 Wiley Periodicals, Inc.
SourceAvailable from: Marilyn J Telen[Show abstract] [Hide abstract]
ABSTRACT: Sickle cell disease (SCD) is the most commonly inherited hemoglobinopathy in the United States. Blood transfusion is a critical part of the multidisciplinary approach necessary in the management of SCD; however, blood transfusions are not without complications. The successful use of transfusion as a treatment strategy in SCD requires the critical review and knowledge of transfusion methods, generally accepted indications, clinical situations in which transfusion generally is not considered, the selection of blood products, and strategies to prevent transfusion-related complications.Hematology/Oncology Clinics of North America 11/2005; 19(5):803-26, v-vi. DOI:10.1016/j.hoc.2005.07.002 · 2.07 Impact Factor
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ABSTRACT: To study the effects of transfusion on the clinical course and oxygenation indexes of children with sickle cell disease and acute chest syndrome. 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. 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. Blood transfusion, even simple transfusion of packed erythrocytes, significantly improves oxygenation in children with acute chest syndrome and is a valuable adjunct to therapy.Journal of Pediatrics 01/1996; 127(6):901-4. DOI:10.1016/S0022-3476(95)70025-0 · 3.74 Impact Factor
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ABSTRACT: Sickle cell disease (SCD) is characterized by recurrent episodes of vaso-occlusion, resulting in tissue ischemia and end-organ damage. Inflammation is critical to the pathogenesis of vaso-occlusion and has been associated with SCD-related morbidity and mortality. Despite the impact of inflammation, no directed anti-inflammatory therapies for the treatment or prevention of vaso-occlusive events currently exist. Among individuals with SCD, asthma is a comorbid inflammatory condition that increases the risk of pain episodes, acute chest syndrome and death. Inflammation associated with asthma could augment the proinflammatory state of SCD, increasing episodes of vaso-occlusion. Leukotrienes are inflammatory mediators that play a prominent role in the pathogenesis of asthma and have been associated with SCD-related morbidity. Targeting inflammatory mediators, such as leukotrienes, is a promising approach for the development of novel therapies for the treatment of SCD. This review will examine the relationship between inflammation and vaso-occlusion, with particular focus on the leukotriene pathway.Expert Review of Hematology 02/2009; 2(1):57-68. DOI:10.1586/17474086.2.1.57 · 2.38 Impact Factor