No abstract

Red 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. A transient reduction in the platelet count by 61% was observed after the procedure. This was not associated with any haemorrhagic complications. Despite exposure to large volumes of blood, the alloimmunisation rate was only 0.027/100 units of red cells. The absence of any iron loading was confirmed by serial Ferriscans, performed over a number of years. However, patients with advanced chronic kidney disease showed evidence of iron loading due to reduced innate haemopoiesis and were subsequently switched to simple transfusions. A total of 59% of patients were on regular automated red cell exchange with a history of recurrent painful crises. A total of 77% responded clinically, as evidenced by at least a 25% reduction in their emergency hospital attendance for pain management. The clinical response was gradual and increased the longer patients stayed on the program. The earliest sign of clinical response was a reduction in the length of stay when these patients were hospitalised, indicating that a reduction in the severity of crises precedes the reduction in their frequency. Automated red cell exchange also appeared to be beneficial for patients with recurrent leg ulcers and severe, drug resistant stuttering priapism, while patients with pulmonary hypertension showed a dramatic improvement in their symptoms as well as echocardiographic parameters.

Fig 1. Distribution of answers according to genotype (A), age (B) and gender (C). Vaccination Y/NY, vaccinated already/keen to be vaccinated but not yet received first dose; Vaccination U, uncertain whether to be vaccinated; Vaccination DW, does not want to be vaccinated.

No abstract

Background Red blood cell exchange is the cornerstone of the management for acute complications of sickle cell disease. It improves anaemia and improvesperipheral tissue oxygen delivery while at the same time reduces the proportion of circulating sickle erythrocytes. Even though automated red cell exchange is very effective in rapidly lowering the Hb S level, 24‐h availability is currently not feasible for most specialist centres including our own. Objective Here, we describe our experience using both automated and manual red cell exchange for the management of acute sickle cell complications. Methods Eighty‐six such episodes have been recorded between June 2011 and June 2022 comprising of 68 episodes of automated and 18 episodes of manual red cell exchange. Results The post procedure Hb S/S + C level was 18% after automated and 36% after manual red cell exchange. The platelet count dropped by 41% and 21% after automated and manual red cell exchange respectively. The clinical outcomes including need for organ support, duration of stay in the intensive care unit and overall length of hospitalisation was comparable between the two groups. Conclusion In our experience, manual red cell exchange is a safe and effective alternative to an automated procedure that can be used while specialist centres are building up their capacity to offer automated red cell exchange for all patients requiring the intervention.