Von Willebrand's disease differs from classic hemophilia by its usually milder manifestations, prolonged bleeding time, predominance of mucous membrane bleeding, and autosomal inheritance.
A review of the Henry Ford Hospital experience from 1959 to 1969 revealed 19 well-documented cases and 22 probable cases. The major diagnostic criteria were (1) significant history of abnormal bleeding; (2) reduced factor VIII activity; and (3) increased bleeding time. Supplementary criteria included family history of the disease, abnormal platelet morphological findings, decreased platelet adhesiveness, and positive plasma infusion tests.
These 41 patients underwent a total of 171 surgical procedures. Without specific therapy, 75 procedures were associated with normal bleeding but in 55 it was excessive. On preoperative treatment with adrenocorticosteroids and fresh frozen plasma, in 40 procedures bleeding was normal and in 3 excessive.
Adelman S, Monto RW, Ponka JL. Surgery in von Willebrand's Disease. Arch Surg. 1972;105(2):204–208. doi:10.1001/archsurg.1972.04180080058010
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