Sickle cell disease (SCD) is a big deal, for so many reasons. Dr. Eric Gehrie is here to guide us through the challenges transfusion services face in association with transfusion in sickle cell patients, especially alloimmunization (making antibodies against red cell antigens).Way back in 1910 or so, Dr. James Herrick discovered that a student in his laboratory science course had some funky-looking RBCs, some of which had a shape like a “sickle.” Over the next ten years, more cases of what came to be known as “sickle cell disease” were described, with those patients suffering severe hemolytic anemia, strokes, and attacks of severe pain. Eventually, the specific problem was identified: A mutation leading to a single amino acid substitution (valine for glutamic acid) in the sixth position of the beta-globin portion of their hemoglobin. Such a simple-seeming substitution now has dire consequences for millions of patients around the world, most commonly in those of African ancestry.
Where do RBCs fit in?
Red blood cell transfusion is a mainstay of therapy in patients with SCD. While lifesaving, especially in regard to stroke prevention and treatment, giving red cells to a patient with SCD is often not as simple as just pulling some units off the shelf and transfusing! In this episode, Dr. Gehrie guides us through some of the things that blood bankers should be thinking when we transfuse RBCs to sickle cell patients. In particular, he will help us work through lots of issues surrounding alloimmunization, which is a massive problem for those with SCD.
(History lesson credit: Bunn HF. Chapter 9: Sickle Cell Disease. Pathophysiology of Blood Disorders, 2nd ed.)
In this episode, Dr. Gehrie mentioned one of the challenges facing U.S. blood bankers and clinicians: The high cost of prophylactic extended serologic or genotypic matching for all RBC transfusions to sickle cell patients. Is that an issue in your particular health care system? I especially wonder about those of you outside of the United States. I’d love to hear your thoughts in the comments section below.
Dr. Eric Gehrie is a physician from whom you will be hearing great things in the future! He is currently the Associate Director of Transfusion Medicine and Assistant Professor of Pathology at Johns Hopkins in Baltimore, MD. He did his transfusion medicine fellowship at Yale-New Haven Hospital and was previously the Assistant Director of the Blood Bank and of Apheresis at that hospital. Dr. Gehrie’s clinical and research interests are focused on the safety and efficacy of blood transfusion and therapeutic apheresis. In particular, he is interested in developing algorithms to predict adverse events related to blood transfusion. He is a co-investigator of the National Heart Lung and Blood Institute (NHLBI) supported Recipient Epidemiology and Donor Evaluation Study-III (REDS-III), where he focuses on the impact of blood transfusion on recipient vital signs and pulmonary injury. He also is a co-investigator of a post-marketing surveillance study evaluating the relationship between platelet transfusion and pulmonary injury in hematology/oncology patients. In addition to these efforts, Dr. Gehrie is committed to patient blood management, with a particular emphasis on the development and implementation of evidence-based guidelines for the initiation of blood component transfusion.
Note: After this podcast was recorded, Dr. Gehrie was named as one of the ASCP’s “40 under 40” honorees as recognition of his leadership and achievements!
DISCLAIMER: The opinions expressed on this episode are those of my guest and I alone, and do not reflect those of the organizations with which either of us is affiliated. Neither Dr. Gehrie nor I have any relevant financial disclosures.
- Responder/Non-responder Discussion: Gehrie EA and Tormey CA. The Influence of Clinical and Biological Factors on Transfusion-Associated Non-ABO Antigen Alloimmunization: Responders, Hyper-Responders, and Non-Responders. Transfus Med Hemother 2014;41:420-429 (free access at time of episode release)
- Sickle Cell Alloimmunization Despite Matching: Chou ST et al. High prevalence of red blood cell alloimmunization in sickle cell disease despite transfusion from Rh-matched minority donors. Blood 2013;122:1062-1071.