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Hemoglobin A Clearance in Children with Sickle Cell Anemia on Chronic Transfusion Therapy
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April 17 2018
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Source: Transfusion. 58(6):1363-1371
[PDF-281.00 KB]
Details:
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Alternative Title:Transfusion
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Personal Author:
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Description:BACKGROUND
Chronic transfusion therapy (CTT) for sickle cell anemia (SCA) reduces disease complications by diluting sickle-erythrocytes with hemoglobin A (HbA)-containing erythrocytes and suppressing erythropoiesis. Minor antigen mismatches may result in alloimmunization, but it is unknown if antigen mismatches or recipient characteristics influence HbA clearance post-transfusion.
STUDY DESIGN AND METHODS
Children with SCA on CTT were followed prospectively for 12 months. All patients received units serologically matched for C/c, E/e, and K; patients with prior red blood cell (RBC) antibodies had additional matching for Fya, Jkb, and any previous alloantibodies. Patients’ RBC antigen genotypes, determined by PreciseType Human Erythrocyte Antigen (HEA), RHCE and RHD BeadChip assays, were compared to genotypes of transfused RBC units to assess for antigen mismatches. Decline in HbA (ΔHbA) from post-transfusion to the next transfusion was calculated for each transfusion episode.
RESULTS
Sixty patients received 789 transfusions, 740 with ∆HbA estimations, and 630 with donor HEA genotyping. In univariate mixed model analysis, ΔHbA was higher in patients with past RBC antibodies or splenomegaly and lower with in patients with splenectomy. RBC antigen mismatches were not associated with ∆HbA. In multivariate linear mixed effects modeling, ΔHbA was associated with RBC antibodies (2.70 vs. 2.45 g/dL/28 days, p=0.0028), splenomegaly (2.87 vs. 2.28 g/dL/28 days, p=0.019), and negatively associated with splenectomy (2.46 vs. 2.70 g/dL/28 days, p=0.011). CONCLUSIONS: HbA decline was increased among SCA patients with prior immunologic response to RBC antigens and decreased among those with prior splenectomy, demonstrating that recipient immunologic characteristics influenced the clearance of transfused RBC.
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Pubmed ID:29664198
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Pubmed Central ID:PMC6021219
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Volume:58
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Issue:6
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