Hemophagocytic lymphohistiocytosis is associated with deficiency and closed conformation of ADAMTS-13

CONCLUSION: Our study reported that ADAMTS-13 conformation is closed in HLH and provides an indirect proof that plasmin is not able to massively degrade ADAMTS-13. Further studies on glycosylation and citrullination profiles of ADAMTS-13 are needed to understand their role in HLH.PMID:38371335 | PMC:PMC10869956 | DOI:10.1016/j.rpth.2023.102292

Source: Thrombosis and Haemostasis - February 19, 2024 Category: Hematology Authors: Am élie Launois Sandrine Valade Eric Mariotte Lionel Galicier Elie Azoulay Elien Roose Karen Vanhoorelbeke Agn ès Veyradier B érangère S Joly Source Type: research