[DDAVP administration in a case of congenital combined factor V and factor VIII deficiency].
Combined deficiency of factor V and factor VIII, a rare bleeding disorder, was found in a 43 year-old male. He had often presented manifestations of easy bruising since childhood, but none of his family had shown evidence of a bleeding tendency. We examined him and his family as far as we could and his abnormality of blood coagulation was apparent, but the members of his family were normal. The prothrombin time and activated partial thromboplastin time of this patient were prolonged, but his thrombin time was normal. Factor V and factor VIII coagulant activity were low, but von Willebrand factor antigen and activity (ristocetin cofactor activity) levels were normal. Protein C and Protein C inhibitor antigen and activity levels were also found to be normal. Following 1-deamino-8-D-arginine vasopressin (DDAVP) injection, he had immediate increases in factor VIII coagulant activity, but both von Willebrand factor antigen, activity levels and factor V coagulant activity remained low. Moreover, there was no rapid decline in factor VIII complex activity. These findings suggest that the endogenous factor VIII in this patient is metabolized normally and that at least the deficiency of factor VIII does not result from accelerated degradation in plasma.
Takai
Y
Y
Hayashi
H
H
Ishimaru
F
F
Fujita
T
T
Tsurumi
N
N
Tsuda
T
T
Kimura
I
I
D000328:Adult; D001777:Blood Coagulation / Q000187:drug effects; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use*; D005166:Factor V Deficiency / Q000151:congenital / Q000188:drug therapy*; D006467:Hemophilia A / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D008297:Male
D003894:Deamino Arginine Vasopressin