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.

Effect of transfusion therapy on cerebral vasculopathy in children with sickle-cell anemia.

This retrospective study assessed the long-term effect of transfusional exchange therapy on MRA/MRI abnormalities in 24 homozygous sickle-cell anemia (HbSS) children presenting with abnormal brain MRA. The median time elapsed from baseline to last available MRA was 29 months. Follow-up MRAs showed improvement, stabilization or worsening of cerebrovascular lesions in 11, 6 and 7 patients respectively. Complete normalization of MRA was observed in 6 patients within a mean time of 1.4 years, but stenosis recurred at the same location in the 4 patients in whom transfusion therapy was discontinued. Baseline severe stenosis/occlusion of large cerebral arteries and occurrence of moyamoya syndrome were significantly associated with an absence of improvement of the cerebral vasculopathy. These data emphasize the heterogeneity of the course of cerebrovasculopathy in SS children receiving chronic transfusion. Further studies are needed to determine whether different therapeutic approaches have to be considered according to these different evolutive patterns in SS children.

Assessment of leucoreduction of sickle cell trait blood: quality of the filtered product.

BACKGROUND: With the implementation of universal leucoreduction of blood components in several industrialised countries, the problems associated with leucocyte filtration of sickle cell trait blood have been reconsidered. In this study, we assessed the use of high performance filters for leucoreduction of packed red blood cells donated from subjects with sickle cell trait and evaluated the incidence and recurrence of altered red blood cell filterability. MATERIALS AND METHODS: Twenty-one volunteer donors with HbAS were compared to 21 donors with HbAA selected at random. The main parameters analysed were residual white blood cell count and post-filtration haemolysis. Filtration times, flow, volume and haemoglobin loss of the packed red blood cells were also determined. RESULTS: In all, 33% of HbAS red blood cell units with slow flow and prolonged filtration time had high residual white blood cell counts. In 7.7% of cases, despite flow through the filter, the units were not leucoreduced properly. Haemoglobin and volume loss were significantly greater in the slow filtration group. Significant post-filtration haemolysis was present in half of the units with high residual white blood cell counts. DISCUSSION: Despite the development of new technology for filtration, the problem of filterability of blood from donors with sickle cell trait is not yet resolved. Altered filterability of blood from sickle cell trait donors cannot be predicted from the donors' characteristics and recurrence of the problem is not observed between donations. Screening blood donors for sickle cell trait to ensure the safety and quality of blood products for transfusion does, therefore, remain a relevant issue.

Severe autoimmune hemolytic anemia caused by a warm IgA autoantibody directed against the third loop of band 3 (RBC anion-exchange protein 1).

BACKGROUND: Autoimmune hemolytic anemia associated with only IgA autoantibodies reacting optimally at 37 degrees C (WAIHA) is exceedingly rare. When identified, warm IgA autoantibodies specificities are usually directed to antigens of the Rh system. However, like IgG autoantibodies, the specificity of the majority of these antibodies is not identified. CASE REPORT: A case of a 3-year-old boy in whom a life-threatening IgA WAIHA occurred suddenly is reported. Following initial RBC transfusions and treatment with steroids at a dose of 3 mg per kg, which was slowly tapered, stabilization to a state of compensated hemolysis was achieved, persisting 4 months before complete resolution. There was no recurrence within a 16-month follow-up. STUDY DESIGN AND METHODS: The standard DAT in a gel column method with anti-IgG and anticomplement reagents was negative. However, the same method with an anti-IgA was strongly positive. RESULTS: The serum and the eluate obtained after acid elution reacted with all normal RBCs tested. Enzymatic treatment of panel RBCs by alpha-chymotrypsin and pronase abolished the reactivity. The reaction was completely inhibited by RBC incubation with four different MoAbs directed against the third extracellular loop of band 3, the RBC anion-exchange protein 1 (AE1), whereas MoAbs against other specificities showed no effect. CONCLUSIONS: This is the first report of an IgA autoantibody directed against the band 3 (AE1) protein and, more specifically, against the third loop. Moreover, this case underlines the importance of including IgA research in the initial diagnostic evaluation when a hemolytic anemia is suspected to be autoimmune and when IgG and complement are not detected on the patient's RBCs.