Participation of Hageman factor dependent pathways in human disease states.

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 C1 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. 