Effectiveness, safety, and cost of partial exchange transfusions in patients with sickle-cell anemia at a sickle cell disease center in sub-Saharan Africa.

The partial exchange transfusions necessary for management of some sickle-cell complications raise the issue of effectiveness in the context of limited resources and inadequate blood safety. This study evaluated the effectiveness, safety, and cost of partial exchange transfusions in 39 patients with sickle-cell anemia in Lubumbashi, looking at the patients' age and gender and the tolerability and direct cost of the transfusions. Excel and SPSS 18 were used for data entry and analysis. Chi2 and Fisher exact tests were used for comparisons. A P-value ≤ 5% was considered statistically significant. The average age of patients was 8.6 ± 6.4 years, and the majority were girls. The most frequent indications were stroke, severe infections, severe vasooclusive crises, and acute chest syndrome. Partial exchange transfusions were effective in improving hemoglobin and hematocrit as well as the percentage of HbS. No acute accident was observed during any partial exchange transfusion; one anti-Kell alloimmunization and 2 cases of iron overload were observed. The annual cost of partial exchange transfusions per patient requiring (and able to afford) regular treatment was US $ 3,345 without iron chelation and more than US $ 5000 with chelation. Partial exchange transfusions are effective and tolerated, but financially inaccessible to the majority of our sickle cell patients. Thus, an assessment is needed of the economic burden of sickle cell complications that require partial exchange transfusions in the context of countries with limited financial resources.

Comparison of automated erythrocytapheresis versus manual exchange transfusion to treat cerebral macrovasculopathy in sickle cell anemia.

BACKGROUND: Chronic exchange transfusion is effective for primary and secondary prevention of stroke in children with sickle cell anemia (SCA). Erythrocytapheresis is recognized to be the most efficient approach; however, it is not widely implemented and is not suitable for all patients. The aim of our study was to compare automated exchange transfusion (AET) with our manual method of exchange transfusion and, in particular, to evaluate the efficacy, safety, and cost of our manual method. STUDY DESIGN AND METHODS: Thirty-nine SCA children with stroke and/or abnormal findings on transcranial Doppler were included in the study. We retrospectively analyzed 1353 exchange sessions, including 333 sessions of AET and 1020 sessions of manual exchange transfusion (MET). RESULTS: Both methods were well tolerated. The median decrease in hemoglobin (Hb)S per session was 21.5% with AET and 18.8% with our manual method (p < 0.0001) with no major increase in red blood cell consumption. Iron overload was well controlled, even with the manual method, with a median (interquartile range) ferritin level of 312 (152-994) µg/L after 24 months of transfusions. The main differences in annual cost relate to equipment costs, which were 74 times higher with the automated method. CONCLUSION: Our study shows that continuous MET has comparable efficacy to the automated method in terms of stroke prevention, decrease in HbS, and iron overload prevention. It is feasible in all hospital settings and is often combined with AET successively over time.

Efficacy, safety and cost-efficiency of using an alternative technique for automated exchange transfusion in pediatric patients with sickle cell disease.

BACKGROUND: Sickle cell disease (SCD) patients suffer complications requiring simple and/or exchange transfusion. In 1999 we developed an automated exchange technique using infusion pumps and vascular catheters (IV Pump Method). OBJECTIVE: To prove that IV Pump Method is cost-efficient, and as safe and effective as automated cell separators. METHODS: Retrospective chart review of SCD patients requiring exchange transfusion admitted to PICU from 2003-2009. Evaluated method used, complications, costs, and Hemoglobin S% (HgS%) change, excluding patients not requiring exchange transfusion. RESULTS: Cost-reduction with IV Pump Method is around $1000. Average HgS% reduction using IV Pump Method was 30.3 vs. 28.8 in Blood Cell Separator group (p = 0.84). We had no complications or mortalities, with the majority of patients being male (p = 0.03) and on the oldest age group (11-19 y/o) for both methods. CONCLUSION: The IV Pump Method is a safe, effective, and cost-efficient alternative to perform exchange transfusion.

Exchange transfusion: a low-cost alternative for severe childhood Guillain-Barré syndrome.

The high cost and nonavailability of plasmapheresis and intravenous immunoglobulin are prohibitive for the treatment of Guillain-Barré syndrome in resource-poor settings. Exchange transfusion can be an alternative therapy for severe disease in children. The effectiveness of exchange transfusion was evaluated in nine children (median age 6 years) with severe Guillain-Barré syndrome (functional disability score >/= 4). All patients had lower limb weakness for a median duration of 4 days (range 2-16 days). Upper limb weakness (n = 8), respiratory involvement (n = 7), pooling of oral secretions (n = 3), and facial palsy (n = 1) were other motor signs. Four children developed respiratory failure; three were ventilated. Other clinical features included leg pains, meningismus and Lasàgue's sign, backache, excessive sweating, and hemodynamic instability. Two children had albuminocytologic dissociation on cerebrospinal fluid analysis. Nerve conduction velocity testing was done in three patients; two had suggestive findings. One course of exchange transfusion could be performed in seven patients. Six children (86%) responded well with one or more of the following: a halt in progressive motor weakness, improvement in leg pains, meningismus, and straight-leg raising within 24 to 48 hours; one could be weaned off the ventilator by 60 hours. All had improvement in motor power of at least one muscle group by one grade within 3 to 7 days. Two patients died, whereas five (71% of the treatment group) were discharged and could walk independently by 4 months. Two untreated ones had died. Exchange transfusion seems to be a safe and effective alternative for severe Guillain-Barré syndrome. It should be offered whenever intravenous immunoglobulin or plasmapheresis is not available or affordable. Replacement of immunosaturated red cells, removal of activated T cells and monocytes, and transient thrombocytopenia are possible explanations for its effectiveness in addition to removal of pathogenic autoantibodies, immune complexes, cytokines, and complements.

Use of erythrocytapheresis in the treatment of patients with sickle cell anemia.

We performed exchange transfusions, utilizing the technique of automated erythrocytapheresis, for the treatment of patients with sickle cell anemia. In an attempt to determine guidelines for the use of erythrocytapheresis, we studied the use of this procedure in three distinct clinical situations in nine patients with sickle cell disease. Patients with dangerous complications of sickle cell disease such as acute respiratory distress and priapism responded well to erythrocytapheresis, showing marked improvement within 24-48 hours. Patients with prolonged painful vasoocclusive crises showed only variable improvement after erythrocytapheresis therapy, insufficient to justify exposing the patient to the risks of the procedure. Patients treated to decrease the frequency of painful crises demonstrated no prolongation in symptom-free intervals between crises. Therefore, erythrocytapheresis has its main value in the management of acute, dangerous complications of sickle cell disease.