Sickle Cell Disease

World Sickle Cell Day 2022

The 19th of June has been designated as World Sickle Cell Awareness Day, the purpose of which is to enhance public education and understanding of sickle cell disease, as well as the challenges faced by patients and their families.

Sickle cell disease (SCD) refers to a group of inherited medical diseases that affect red blood cells. People with sickle cell disease generate abnormally shaped red blood cells, which can create complications since they do not live as long as healthy blood cells and can clog blood arteries.

Healthy red blood cells are spherical and flow through tiny blood channels to transport oxygen to all regions of the body. SCD causes red blood cells to become hard and sticky, like a C-shaped farm tool known as a “sickle.” The sickle cells die prematurely, resulting in a chronic lack of red blood cells. Furthermore, as they pass through small blood arteries, they become caught and obstruct the blood flow. This can result in discomfort as well as other major issues such as infection, acute chest syndrome, and stroke.

In the paper referenced below, researchers have used soft x-ray tomography (SXT), correlated with light microscopy, to understand the variation in cellular morphology, in terms of volume and in the number of protrusions per cell of sickled red blood cells. The use of SXT in this study has provided the three-dimensional resolution necessary to unambiguously classify red blood cells beyond the binary categories of ‘biconcave disc’ and ‘aberrant’ that are commonly used to describe sickle RBCs in light microscopy.

The above-referenced SXT research was carried out at a synchrotron beamline, where access to the soft x-ray microscope is limited. The authors note that a lab-based soft x-ray microscope would have allowed them to image a larger sample of cells and to produce results with a higher level of statistical confidence. SiriusXT’s lab-based soft x-ray microscope, the SXT-100, was not available at the time this research was carried out, however, with its pending launch in 2022, this valuable imaging modality becomes available to every researcher, who wants to undertake longer studies or at a faster turnaround than is available at the synchrotron-based soft x-ray microscopes.

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Visualizing red blood cell sickling and the effects of inhibition of sphingosine kinase 1 using soft X-ray tomography

Michele C.Darrow, Carolyn A. Larabell,