Understanding Red Blood Cell Health and the Management of Sickle Cell Disease - Episode 2

RBC Biochemistry, Form, and Function

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Nirmish Shah, MD, discusses the biochemical pathways within RBCs and their importance in facilitating a healthy RBCs form and function.

Biree Andemariam, MD: I think we all agree with you. Nirmish, do you have anything to add to that? Specifically, start introducing our audience to some of the biochemical pathways that contribute to healthy red blood cells’ form and function, including where kinases are and ATP [adenosine triphosphate] but also touching on hemoglobin. What are the different subtypes and their effect on this transport process?

Nirmish Shah, MD: Whenever you have a bunch of hematologists in a room or on a panel, we get excited about talking about red blood cells because they’re pretty complex. There are a lot of moving parts. Red blood cell health is rightfully becoming more of a hot topic. What is red blood cell health? How do we improve on red blood cell health? There are different ways to do this, but for a patient with sickle cell [disease], [it means having] red blood cells that do not sickle, are malleable, are able to get to where they need to go and deliver oxygen appropriately. There are a lot of parts to what it should be doing.

How do we improve on that in sickle cell disease? One method is to concentrate on the oxygen dissociation curve. As mentioned by Elna [Saah], the job is to deliver the oxygen when it’s needed. In sickle cell disease, that curve is inappropriately shifted to the right. We need to focus on methods to try to shift it back to where hemoglobin A is, where the curve is naturally occurring. One way to do that is to focus on the pyruvate kinase receptor. That decreases 2,3 DPG [diphosphoglycerate], which is an effective way of shifting that curve to the left. Focusing on 2,3 DPG and pyruvate kinase receptor agonists is 1 method of trying to focus on not sickling and shifting the curve, delivering oxygen and ultimately red cell health.

Transcript edited for clarity