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Graft-derived VWF drives platelet activation and thrombocytopenia during porcine liver xenotransplantation to brain-dead human recipients - PubMed

a day ago
  • #xenotransplantation
  • #VWF
  • #thrombocytopenia
  • Genetically engineered porcine livers are being developed as a bridge therapy for acute liver failure.
  • Severe thrombocytopenia is a major limitation in porcine liver xenotransplantation.
  • Platelet kinetics were studied in human decedents undergoing cross-circulation with transgenic porcine livers.
  • Rapid platelet clearance (80%-90%) was observed, inconsistent with marrow suppression or hypersplenism.
  • Antibody and complement inhibition did not improve thrombocytopenia.
  • Porcine von Willebrand factor (pVWF) mediated platelet activation, similar to human Type IIb von Willebrand disease.
  • Caplacizumab, an anti-VWF nanobody, blocked pVWF-mediated platelet activation ex vivo.
  • In a fourth decedent, caplacizumab prevented aberrant platelet activation but full recovery was limited by pre-existing DIC.
  • Thrombocytopenia in xenotransplantation is primarily driven by pVWF-mediated platelet activation, not immune or splenic mechanisms.
  • Targeted VWF blockade with caplacizumab may improve the safety of porcine liver support in acute liver failure.