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

Authors

  • Patricia M. Raciti,

    1. Department of Pathology and Cell Biology, Columbia University Medical Center-NewYork-Presbyterian Hospital, New York, New York
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  • Richard O. Francis,

    1. Department of Pathology and Cell Biology, Columbia University Medical Center-NewYork-Presbyterian Hospital, New York, New York
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  • Patrice F. Spitalnik,

    1. Department of Pathology and Cell Biology, Columbia University Medical Center-NewYork-Presbyterian Hospital, New York, New York
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  • Joseph Schwartz,

    1. Department of Pathology and Cell Biology, Columbia University Medical Center-NewYork-Presbyterian Hospital, New York, New York
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  • Jeffrey S. Jhang

    Corresponding author
    • Department of Pathology and Cell Biology, Columbia University Medical Center-NewYork-Presbyterian Hospital, New York, New York
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Correspondence to: Jeffrey S. Jhang, M.D., College of Physicians and Surgeons of Columbia University, 180 Fort Washington Avenue, Harkness Pavilion 4–414, New York, NY 10032, USA. E-mail: jj222@columbia.edu

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. J. Clin. Apheresis 28:325–329, 2013. © 2013 Wiley Periodicals, Inc.

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