Acquired hemoglobin variants and exposure to glucose‐6‐phosphate dehydrogenase deficient red blood cell units during exchange transfusion for sickle cell disease in a patient requiring antigen‐matched blood

Abstract

Red blood cell exchange (RBCEx) is frequently used in the management of patients with sickle cell disease (SCD) and acute chest syndrome or stroke, or to maintain target hemoglobin S (HbS) levels. In these settings, RBCEx is a category I or II recommendation according to guidelines on the use of therapeutic apheresis published by the American Society for Apheresis. Matching donor red blood cells (RBCs) to recipient phenotypes (e.g., C, E, K-antigen negative) can decrease the risk of alloimmunization in patients with multi-transfused SCD. However, this may select for donors with a higher prevalence of RBC disorders for which screening is not performed. This report describes a patient with SCD treated with RBCEx using five units negative for C, E, K, Fya, Fyb (prospectively matched), four of which were from donors with hemoglobin variants and/or glucose-6-phosphate dehydrogenase (G6PD) deficiency. Pre-RBCEx HbS quantification by high performance liquid chromatography (HPLC) demonstrated 49.3% HbS and 2.8% hemoglobin C, presumably from transfusion of a hemoglobin C-containing RBC unit during a previous RBCEx. Post-RBCEx HPLC showed the appearance of hemoglobin G-Philadelphia. Two units were G6PD-deficient. The patient did well, but the consequences of transfusing RBC units that are G6PD-deficient and contain hemoglobin variants are unknown. Additional studies are needed to investigate effects on storage, in-vivo RBC recovery and survival, and physiological effects following transfusion of these units. Post-RBCEx HPLC can monitor RBCEx efficiency and detect the presence of abnormal transfused units.

Ethnicity, sex, and age are determinants of red blood cell storage and stress hemolysis: results of the REDS-III RBC-Omics study

“…16,17 Functionally relevant RBC polymorphisms are anticipated to be high in donors with racial origin or admixture from malaria-endemic regions, such as equatorial Africa, Asia, and South and Central America. 18 In these donors, certain conserved RBC mutations that historically conferred resistance against malaria may impact RBC functional characteristics and be disadvantageous with regard to cold storage.…”

Glucose‐6‐phosphate dehydrogenase deficiency in Italian blood donors: prevalence and molecular defect characterization

“…The criteria for the selection of blood donors do not exclude the presence of this genetic disorder, and it was observed that specific donor RBC units are more likely to haemolyse during storage . Transfusion outcomes of RBC units from G6PD‐deficient donors have been recently discussed especially for high risk patients as premature infants and neonates, and patients with sickle cell disease (SCD) submitted to multiple transfusions . Moreover, checking for the presence of G6PD deficiency in blood donors or in RBC units was suggested in the regions with high frequency of G6PD deficiency .…”

Acquired hemoglobin C secondary to transfusion with antigen‐matched red blood cells

“…Raciti et al [1] recently described a patient with sickle cell disease requiring chronic transfusion support, who was passively transfused with hemoglobin C (HgbC), hemoglobin G-Philadelphia, and glucose-6phosphate dehydrogenase deficient RBC units all of which were antigen-matched to reduce the likelihood of alloimmunization. We would like to share our experience with a similar patient.…”

“…As mentioned by Raciti et al [1], we too considered the consequences of antigen-matching for this patient. Donors negative for K1, S, and Fy a antigens are significantly more common among black donors (60% versus 15% white) [2], thus increasing the likelihood of transfusion with HgbC.…”