Korean J Hematol.
1997 Nov;32(3):423-427.
Two Cases of Congenital Factor VII Deficiency with Family Study
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- 1Department of Clinical Pathology, Inha University College of Medicine, Inchon, Korea.
Abstract
- Congenital factor VII deficiency is a rare coagulation disorder transmitted in autosomal recessive pattern and is characterized by prolonged prothrombin time with normal activated partial thromboplastin time. It is confirmed by specific factor VII assay. Heterozygotes are generally asymptomatic and homozygotes may present variety of bleeding symptom. But heterozygotes are not always asymptomatic and that patients should receive replacement of factor VII for their operation or abnormal bleeding. We experienced 2 cases of congenital factor VII deficiency diagnosed by prolonged prothrombin time and factor VII assay in routine preoperative evaluation. One was a 27-year-old female who seemed to be an asymptomatic heterozygote underwent orthopedic surgical procedure successfully without abnormal bleeding after receiving fresh frozen plasma. Another was a 18-year-old male who also seemed to be a heterozygote including his family had bleeding symptom such as epistaxis.
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