Lung  Transplantion Correct Von Willebrand Deficiency


I was close to some of the early work on attempts to identify the site of factor VIII synthesis (1-3).  Transplanted organs included the liver, spleen, and kidney.  Equivocal results were obtained which can now be understood with the identification of the endothelial cell as site of synthesis (4-6).  However, the identification of the lung as a site of synthesis for von Willebrand factor, a traveling companion for factor VIII came a surprise.   Ferés (1) and coworkers in Spain announce that lung transplantation corrected a mild case of von Willebrand Disease.


References


1.  Norman, J.C., Lambilliotte, J.P., Kojima, Y., and Sise, H.S.(1967), Antihemophilic factor release by perfused liver and spleen: relationship to hemophilia, Science  158, 1060-1061.
2.   Peacock, E.E.,Jr., Webster, W.P.,  Penick, G.D., Madden, J.W., and Hutchin, P.(1969), Transplantation of the spleen, Transplant.Proc. 1, 239-245.
3.  Webster, W.P., Zukoski, C.F., Hutchin, P., et al.(1971), Plasma factor VIII synthesis and control as revealed by canine organ transplantation, Am.J.Physiol. 220, 1147-1154.
4.  Everett, L.A., Cleuren, A.C.A., Khoriaty, R.N., and Ginsberg, D. (2014), Murine coagulation factor VIII is synthesized in endothelial cells, Blood 123, 3697-3705.
5.  Shahani, T., Covens, K., Lavend'homme, R., et al.(2014), Human liver sinusoidal endothelial cells but not hepatocytes contain factor VIII, J.Thromb.Haemost. 12, 36-42.
6.   Fahs, S.A., Hille, M.T., Shi, Q., Weiler, H., and Montgomery, R.R.(2014), A conditional knockout mouse model reveals endothelial cells as the principal and possibly exclusive source of plasma factor VIII, Blood 123, 3706-3713.
7.  Forés, R., Lario, A., Gil, S., et al., Von Willebrand factor deficiency corrected by lung transplantation, Transplantation, in press, 2015