AI Article Synopsis
- * Acute chest syndrome (ACS) is a severe complication of SCD that contributes significantly to patient mortality and can develop from VOE.
- * Recent research shows that a P-selectin monoclonal antibody, specifically a fusion molecule called TSGL-Ig, can reduce lung vaso-occlusion in SCD mice, demonstrating its potential to prevent both VOE and ACS in SCD patients.
Article Abstract
Sickle cell disease (SCD) is a monogenic disorder estimated to affect more than three million people worldwide. Acute systemic painful vaso-occlusive episode (VOE) is the primary reason for emergency medical care among SCD patients. VOE may also progress to acute chest syndrome (ACS), a type of acute lung injury and one of the primary reasons for mortality among SCD patients. Recently, P-selectin monoclonal antibodies were found to attenuate VOE in SCD patients and lung vaso-occlusion in transgenic humanized SCD mice, highlighting the therapeutic benefit of P-selectin inhibition in SCD. Here, we use quantitative fluorescence intravital lung microscopy (qFILM) to illustrate that tandem P-selectin-glycoprotein ligand-immunoglobulin (TSGL-Ig) fusion molecule containing four P-selectin binding sites, significantly attenuated intravenous (IV) oxyhemoglobin triggered lung vaso-occlusion in SCD mice. These findings highlight the therapeutic potential of TSGL-Ig in preventing VOE and ACS in SCD.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7237323 | PMC |
| http://dx.doi.org/10.1016/j.exphem.2020.03.002 | DOI Listing |
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