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Four Cases of Hemophilia B
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HOME > J Pathol Transl Med > Volume 10(1); 1976 > Article
Etc Four Cases of Hemophilia B
Journal of Pathology and Translational Medicine 1976;10(1):169-176
DOI: https://doi.org/
Department of Laboratory Medicine, College of Medicine, Seoul National University, Seoul, Korea
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Hemophilia B is a hemorrhagic disease characterized by a deficiency of factor Ⅸ activity and a sex-linked recessive mode of inheritance, and is clinically indistinguishable from hemophilia A. Four cases of proven hemophilia B are presented with a brief review of the literature. The first case (M 1-l/2 yr.) was associated with idiopathic thrombocytopenic purpura, and plasma factor Ⅸ level was 0.3% of normal. The second (M 20yr.) and the third (M 24yr.) cases were siblings, with 1.0% and 0.7% of factor Ⅸ activity. Family study of these second and third cases showed that their father had subhemophilic level of factor Ⅸ activity (35%), and their mother, an obligatory carrier, and their sister, a potential carrier, had reduced factor Ⅸ levels, 48% and 35% respectively. The fourth case (M 18 yr.) had 3.2% of factor Ⅸ activity. The authors believe that this is the first report of factor assay-proven cases of hemophilia B in the Korean literature.

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