Korean J Hematol.
1997 Nov;32(3):419-422.
A Case of Factor VII Deficiency with Normal Factor VII Antigen Level
- Affiliations
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- 1Department of Clinical Pathology, Catholic University Medical College, Seoul, Korea.
- 2Department of Internal Medicine, Catholic University Medical College, Seoul, Korea.
Abstract
- Factor VII (FVII) deficiency is a rare hereditary disorder with a reduced FVII:C activity, which is transmitted by an autosomal recessive gene with variable expressions and a high penetrance. FVII deficiency has different antigen levels, normal or reduced, and has various symptoms. We report on a 26 year-old male patient with pleurisy who showed hematuria and prolonged prothrombin time. FVII:C was 4% and FVII:Ag level was 80% (within normal range). The inheritance of this case is unclear. The FVII:C, FVII:Ag, and prothrombin time of his father and mother's sister were within normal ranges. This is the first proven case of FVII deficiency with normal FVII:Ag level in Korea.
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