Ektacytometry Analysis of Post-splenectomy Red Blood Cell Properties Identifies Cell Membrane Stability Test as a Novel Biomarker of Membrane Health in Hereditary Spherocytosis

• DOI: 10.3389/fphys.2021.641384
• Publication Date: 2021-03-25T05:34:03Z
• Authors: M. C. Berrevoets; J. Bos; R. Huisjes; T. H. Merkx; B. A. van Oirschot; W. W. van Solinge; J. W. Verweij; M. Y. A. Lindeboom; E. J. van Beers; M. Bartels; R. van Wijk; M. A. E. Rab
• Keywords: Hereditary spherocytosis; Spherocytosis

Hereditary spherocytosis (HS) is the most common form of hereditary chronic hemolytic anemia. It caused by mutations in red blood cell (RBC) membrane and cytoskeletal proteins, which compromise integrity, leading to vesiculation. Eventually, this leads entrapment poorly deformable spherocytes spleen. Splenectomy a procedure often performed HS. The clinical benefit results from removing primary site destruction, thereby improving RBC survival. But whether changes properties contribute splenectomy unknown. In study we used ektacytometry investigate longitudinal effects on five well-characterized HS patients at four different time points case-control cohort 26 patients. Osmotic gradient showed that resulted improved intracellular viscosity (hydration state) whereas total surface area surface-to-volume ratio remained essentially unchanged. stability test (CMST), assesses vitro response shear stress, after splenectomy, RBCs had partly regained ability shed membrane, property healthy RBCs, was confirmed cohort. particular CMST holds promise as novel biomarker reflects health may be asses treatment