Efficacy and Safety of Manual Partial Red Cell Exchange in the Management of Severe Complications of Sickle Cell Disease in a Developing Country

Abstract

Introduction The realization of red cell exchange (RCE) in Africa faces the lack of blood, transfusion safety, and equipment. We evaluated its efficacy and safety in severe complications of sickle cell disease. Patients and Method Manual partial RCE was performed among sickle cell patients who had severe complications. Efficacy was evaluated by clinical evolution, blood count, and electrophoresis of hemoglobin. Safety was evaluated on adverse effects, infections, and alloimmunization. Results We performed 166 partial RCE among 44 patients including 41 homozygous (SS) and 2 heterozygous composites SC and 1 S/β0-thalassemia. The mean age was 27.9 years. The sex ratio was 1.58. The regression of symptoms was complete in 100% of persistent vasoocclusive crisis and acute chest syndrome, 56.7% of intermittent priapism, and 30% of stroke. It was partial in 100% of leg ulcers and null in acute priapism. The mean variations of hemoglobin and hematocrit rate after one procedure were, respectively, +1.4 g/dL and +4.4%. That of hemoglobin S after 2 consecutive RCE was −60%. Neither alloimmunization nor viral seroconversion was observed. Conclusion This work shows the feasibility of manual partial RCE in a low-resource setting and its efficacy and safety during complications of SCD outside of acute priapism.

Transfusion Practice, Post-Transfusion Complications and Risk Factors in Sickle Cell Disease in Senegal, West Africa.

“…12,16,17 The objective of BT in SCD is not only correct chronic anemia but also to decrease HBS level and therefore to prevent or treat SCD complications whereas in case, of a simple or a transfusion exchange, either in an acute context or during a transfusion program. 3,6 Iterative transfusions are exposed to RCA, infectious and iron overload which pose a great risk of morbidity and mortality for SCD patients. 4,5 In this study, simple transfusion was performed in the majority of cases and few patients had a chronic transfusion program.…”

“…3,4 It exists in three methods to treat SCD anemia: simple BT, punctual transfusion exchange, and longterm transfusion exchange. 5,6 Despite the benefits, multi-transfused patients are at an increased risk of complications of red cell alloimmunization, iron overload, and blood-transmitted infections. 7,8,9 The frequency of these complications correlates with RBCs units transfused.…”

What is the effectiveness of surgical and non-surgical therapies in the treatment of ischemic priapism in patients with sickle cell disease? A systematic review by the EAU Sexual and Reproductive Health Guidelines Panel

“…Exchange Blood transfusion has also been used in preventing SCD-related IP episodes. In general, blood exchange therapy was utilized after failure of other conservative measures such as aspiration, irrigation or injection of sympathomimetics 13,15,19,[22][23][24]26,28,34,37,39,46,49,53 . In a study including 10 SCD-related IP patients, exchange transfusion fully controlled priapic episodes in 50% of the patients.…”

“…Several studies demonstrated that blood exchange transfusion is safe 19,26,34,37 . ASPEN syndrome was not reported in most studies 19,22,26 but one study reported post-blood exchange mental confusion in 7 patients, which resolved several days after without any long-term sequalae 34 .…”

Vascular access for red blood cell exchange

“…Data are sparse on the utility of RBC exchange for vaso‐occlusive pain crises. One group in a developing country noted that manual partial RBC exchange was tolerated and at least partially successful in reducing symptoms for both acute and chronic complications of SCD, including persistent vaso‐occlusive crises …”