Background: Posttransfusion hyperhemolysis syndrome is a rare but life-threatening form of delayed hemolytic transfusion reaction with lysis of both transfused and autologous red cells, seen predominantly in patients with sickle cell disease. Macrophage activation is thought to play a major role in its pathophysiology. Standard treatment is with intravenous immunoglobulin and steroids but refractory cases pose a major clinical problem.
View Article and Find Full Text PDFRed cell transfusion represents one of the cornerstones of the chronic management of sickle cell disease, as well as its acute complications. Automated red cell exchange can rapidly lower the number of circulating sickle erythrocytes, without causing iron overload. Here, we describe our experience, having offered this intervention since 2011.
View Article and Find Full Text PDFSickle cell disease is characterised by recurrent painful crises often leading to hospitalisation. During the COVID-19 pandemic, it was important to try to reduce the need for hospital admission for these high-risk patients while at the same time ensuring that hospital avoidance did not put them at risk of deterioration from disease-related complications. In the 3-month period between March and May 2020, there was a significant reduction in the number of hospital admissions as well as mean length of stay compared with the mean figures over the same months in the preceding 5 years (2015-19), with an overall reduction in inpatient days of 77%.
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