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
Article first published online: 1 MAR 2013
Copyright © 2013 Wiley Periodicals, Inc.
Journal of Clinical Apheresis
Volume 28, Issue 4, pages 325–329, August 2013
How to Cite
Raciti, P. M., Francis, R. O., Spitalnik, P. F., Schwartz, J. and Jhang, J. S. (2013), 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. J. Clin. Apheresis, 28: 325–329. doi: 10.1002/jca.21255
- Issue published online: 12 AUG 2013
- Article first published online: 1 MAR 2013
- Manuscript Accepted: 9 NOV 2012
- Manuscript Revised: 7 NOV 2012
- Manuscript Received: 5 SEP 2012
- glucose-6-phosphate dehydrogenase deficiency;
- RBC phenotype matching
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. J. Clin. Apheresis 28:325–329, 2013. © 2013 Wiley Periodicals, Inc.