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A preoperative hemostasis study discovered a prolonged activated partial thromboplastin time in a 23-year-old Portuguese Caucasian woman without personal or past family history of hemorrhage or thrombosis. This was corrected by pooled plasma that excluded circulating anticoagulant. Activated partial thromboplastin time was prolonged whatever the activator, particularly ellagic acid, and was not corrected by prolonged kaolin incubation. Levels of factors VIII and XII were normal; factor XI and prekallikrein levels were either moderately low or normal according to activators and defective reagents used. High-molecular-weight kininogen (HMWK) level assessed by coagulation and immunological method was virtually nil. Fibrinolysis activity was normal before and after venous occlusion. The programmed operation was performed without any particular preparation and no complication arose. Family investigation found heterozygous HMWK deficiency in the proposita's father and three of her siblings.
J J Lefrère, and
M H Horellou, and
D Gozin, and
J Conard, and
J Y Muller, and
M Clark, and
J P Soulier, and
M Samama
January 1998,
Ryoikibetsu shokogun shirizu,
J J Lefrère, and
M H Horellou, and
D Gozin, and
J Conard, and
J Y Muller, and
M Clark, and
J P Soulier, and
M Samama
August 1987,
Nihon Ketsueki Gakkai zasshi : journal of Japan Haematological Society,
J J Lefrère, and
M H Horellou, and
D Gozin, and
J Conard, and
J Y Muller, and
M Clark, and
J P Soulier, and
M Samama
December 1988,
[Rinsho ketsueki] The Japanese journal of clinical hematology,
J J Lefrère, and
M H Horellou, and
D Gozin, and
J Conard, and
J Y Muller, and
M Clark, and
J P Soulier, and
M Samama
January 2016,
Haemophilia : the official journal of the World Federation of Hemophilia,
J J Lefrère, and
M H Horellou, and
D Gozin, and
J Conard, and
J Y Muller, and
M Clark, and
J P Soulier, and
M Samama
September 1985,
Nihon Ketsueki Gakkai zasshi : journal of Japan Haematological Society,
J J Lefrère, and
M H Horellou, and
D Gozin, and
J Conard, and
J Y Muller, and
M Clark, and
J P Soulier, and
M Samama
February 2001,
Thrombosis and haemostasis,
J J Lefrère, and
M H Horellou, and
D Gozin, and
J Conard, and
J Y Muller, and
M Clark, and
J P Soulier, and
M Samama
January 1989,
Methods in enzymology,
J J Lefrère, and
M H Horellou, and
D Gozin, and
J Conard, and
J Y Muller, and
M Clark, and
J P Soulier, and
M Samama
January 1992,
Methods in enzymology,
J J Lefrère, and
M H Horellou, and
D Gozin, and
J Conard, and
J Y Muller, and
M Clark, and
J P Soulier, and
M Samama
February 1976,
The Journal of laboratory and clinical medicine,
J J Lefrère, and
M H Horellou, and
D Gozin, and
J Conard, and
J Y Muller, and
M Clark, and
J P Soulier, and
M Samama
March 1990,
Blood,
