Summary
Abnormalities of Hageman factor dependent pathways have been described in a wide variety
of human disease states. Congenital deficiencies of factor XII (Hageman trait) prekallikrein
(Fletcher trait) and high molecular weight kininogen (Williams, Fitzgerald and Flaujeac
traits) although resulting in profound in vitro changes, do not cause in vivo difficulties. In contrast, deficiency of Cl esterase inhibitor (hereditary angioedema)
results in significant morbidity and mortality. Acquired diseases may exhibit decreased
synthesis of these three proteins in cirrhosis and dengue fever. In vivo activation of factor XII initiated pathways occur in septic shock, disseminated or
localized intravascular coagulation, typhoid fever, polycythemia vera, hyperbetalipoproteinemia,
coronary artery disease, nephrotic syndrome, transfusion reactions, hemodialysis and
extracorporeal bypass. Activation of both the intrinsic system and tissue mediators
contribute to the vasomotor phenomena in carcinoid syndrome and postgastrectomy dumping.
Roles for factor XII, prekallikrein and kininogen have been suggested in gouty arthritis,
allergic disorders and cystic fibrosis but the evidence is not yet convincing in these
disorders.
