6533b834fe1ef96bd129d794

RESEARCH PRODUCT

FVIII production by human lung microvascular endothelial cells

Marc JacqueminMarc JacqueminKathelijne PeerlinckKathelijne PeerlinckDirk Van RaemdonckDirk Van RaemdonckFilip RegaFilip RegaRenaud Lavend'hommeRenaud Lavend'hommeMaria Iris HermannsMaria Iris HermannsJean-marie Saint-remyJean-marie Saint-remyArne NeyrinckArne NeyrinckC.j. KirkpatrickC.j. Kirkpatrick

subject

Pulmonary Circulationcongenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtyEndotheliumanimal diseasesImmunologyIn Vitro TechniquesFibrinogenBiochemistryImmunoglobulin GMicrocirculationVon Willebrand factorhemic and lymphatic diseasesInternal medicinevon Willebrand FactormedicineHumansLungFactor VIIILungbiologybusiness.industryMicrocirculationEndothelial CellsCell BiologyHematologyTransplantationKineticsEndocrinologymedicine.anatomical_structureHemostasisReperfusionImmunologybiology.proteinEndothelium Vascularbusinessmedicine.drug

description

While extrahepatic factor VIII (FVIII) synthesis suffices for hemostasis, the extrahepatic production sites are not well defined. We therefore investigated the ability of the human lungs to produce FVIII. Lungs from heart-beating donors who were declined for transplantation were perfused and ventilated in an isolated reperfusion model for 2 hours. A progressive accumulation of FVIII and von Willebrand factor (VWF) was recorded in the perfusion medium in 3 of 4 experiments. By contrast, factor V, fibrinogen, and immunoglobulin G (IgG) levels remained constant during the perfusion period, indicating that the accumulation of FVIII and VWF was not due to diffusion from the intercellular medium into the vascular system. Purified human lung microvascular endothelial cells produced FVIII during at least 2 passages in vitro. Altogether, these data identify the lung endothelial cells as a FVIII production site in humans.

https://doi.org/10.1182/blood-2005-11-4571