title: 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.
authors: Department of Pathology and Cell Biology, Columbia University Medical Center-NewYork-Presbyterian Hospital, New York, NY, USA.
authors: Patricia M
authors: PM
authors: Raciti
authors: Richard O
authors: RO
authors: Francis
authors: Patrice F
authors: PF
authors: Spitalnik
authors: Joseph
authors: J
authors: Schwartz
authors: Jeffrey S
authors: JS
authors: Jhang
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000276:immunology / Q000628:therapy*; D017707:Erythrocyte Transfusion / Q000009:adverse effects*; D004912:Erythrocytes / Q000201:enzymology*; D005078:Exchange Transfusion, Whole Blood / Q000009:adverse effects*; D005260:Female; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000150:complications*; D006454:Hemoglobins / Q000235:genetics*; D006801:Humans
chemical_list: D006454:Hemoglobins
keywords: RBC phenotype matching; alloimmunization; enzymopathy; glucose-6-phosphate dehydrogenase deficiency; hemoglobinopathy