A Japanese Family of Hereditary Deficiency of Plasma Thromboplastin Antecedent （Factor ⅩⅠ）

A Japanese Family of Hereditary Deficiency of Plasma Thromboplastin Antecedent （Factor ⅩⅠ）

Creators Shinohara kenji

Creators Konishi Yasuko

Creators Azuno Yoichi

Creators Yamada Katsunori

Creators Sasaki Hiroko

Creators Kaneko Toshio

Creators Matsumoto Noboru

A Japanese family of hereditary deficiency of plasma thromboplastin antecedent (PTA, factor XI ) was reported. The parents of the propositus were of consanguinous marriage. The levels of plasma factor Xiactivity and anitigen of the homozygotes were less than 1% of normal, and those of heterzygotes were approximately half of normal. The deficiency was transmitted as autosomal recessive trait. The homozygotes had no episodes of troublesome bleedings. The platelets of the propositus contained intrinsic factor XI-like activity as in normal, and the compensatory role of platelet factor XI-like activity for the initiation of the intrinsic pathway of coagulation was suggested.

[ISSN]0513-1812

[NCID]AA00594272