A Case of Factor VII Deficiency with Normal Factor VII Antigen Level.
- Author:
Hi Jeong KWON
1
;
Hwa Sik MOON
Author Information
1. Department of Clinical Pathology, Catholic University Medical College, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Factor VII deficiency;
FVII:C;
FVII:Ag;
Prolonged prothrombin time
- MeSH:
Adult;
Factor VII Deficiency*;
Factor VII*;
Fathers;
Genes, Recessive;
Hematuria;
Humans;
Korea;
Male;
Penetrance;
Pleurisy;
Prothrombin Time;
Reference Values;
Siblings;
Wills
- From:Korean Journal of Hematology
1997;32(3):419-422
- CountryRepublic of Korea
- Language:Korean
-
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.
