[Coagulation and fibrinolysis associated with contact phase in plasma with high molecular weight kininogen deficiency].
Congenital deficiency of high molecular weight kininogen (HMWK), first reported by Saito et al. in 1975, is a rare disorder with homozygous defect of HMWK and only 14 cases have been reported so far. Prolonged partial thromboplastin time and depressed intrinsic fibrinolytic activity in plasma interacted with negatively charged surface were characterized in these patients without any bleeding problem. On the other hand, patients associated with hemorrhagic disorder, such as haemophiliacs, showed a markedly prolonged partial thromboplastin time but normal intrinsic fibrinolytic activity. These results give us a important suggestion on our understanding of why haemophiliacs bleed and why HMWK deficient patients do not. In immunological studies with specific antisera directed against human plasma HMWK and prekallikrein, HMWK in present in normal plasma in free form and a complexed form with prekallikrein, and whole prekallikrein in present in a complexed form with HMWK. This result is associated with diminished level of prekallikrein in plasma of patients with congenital HMWK deficiency. According to above-mentioned our experimental results and some reports, it is speculated that the deficiency of HMWK with normal level of prekallikrein is acquired.
Iijima
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K
Nakamura
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D000328:Adult; D000368:Aged; D001777:Blood Coagulation; D005174:Factor XII / Q000032:analysis; D005260:Female; D005342:Fibrinolysis; D006801:Humans; D007704:Kininogens / Q000032:analysis / Q000172:deficiency*; D008297:Male; D008875:Middle Aged; D010314:Partial Thromboplastin Time; D011288:Prekallikrein / Q000032:analysis
D007704:Kininogens; D005174:Factor XII; D011288:Prekallikrein