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.
Highlights
Red cell transfusion improves tissue oxygen delivery and lowers the proportion of sickle erythrocytes in the circulation, making it a key intervention in both the chronic management of sickle cell disease (SCD) as well as in its acute complications
In this article we describe our experience at the sickle cell and thalassaemia Centre at Homerton Hospital, London, UK, providing regular Automated red cell exchange (a-RCE) as a disease-modifying intervention in our institution since June 2011 and present data on several different aspects of the procedure
Sickle cell nephropathy (SCN) was defined as persistent proteinuria or evidence of chronic kidney disease (CKD); CKD was defined as an estimated glomerular filtration rate of less than 90 mL/min [4]
Summary
Red cell transfusion improves tissue oxygen delivery and lowers the proportion of sickle erythrocytes in the circulation, making it a key intervention in both the chronic management of sickle cell disease (SCD) as well as in its acute complications. Exchange transfusion can be manual when the patient’s blood is removed and replaced by donor red cells and replacement fluid, usually normal saline to maintain isovolaemia. This process is laborious, less effective in reducing the Hb S level, especially in the acute situation and can still lead to a degree of iron overload. Is performed through an apheresis system where the patient’s own red cells are removed and replaced with donor red cells before the blood is returned to the patient This process requires specialist staff and equipment but can rapidly reduce the Hb S level, avoiding iron loading, it is the recommended mode of delivering chronic transfusions for patients with SCD [3]. In this article we describe our experience at the sickle cell and thalassaemia Centre at Homerton Hospital, London, UK, providing regular a-RCE as a disease-modifying intervention in our institution since June 2011 and present data on several different aspects of the procedure
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