Zhang Y, Zhao J Z, Jiang Q, Jiang H, Lu J, Fu H X, Lü M, Xu L P, Zhang X H
Department of Hematology, Peking University People's Hospital, Peking University Institute of Hematology, Beijing Key Laboratory of Hematopoietic Stem Cell Transplantation, Beijing 100044, China.
Zhonghua Yi Xue Za Zhi. 2018 Sep 25;98(36):2920-2924. doi: 10.3760/cma.j.issn.0376-2491.2018.36.009.
To analyze the clinical characteristics of hereditary coagulation factor Ⅴ deficiency (FⅤD) and to improve the diagnosis and treatment ability of hereditary FⅤD. A total of 17 patients with hereditary FⅤD admitted to the Department of Hematology, Peking University People's Hospital from February 2013 to January 2018 were selected, and their clinical characteristics, laboratory examination, treatment and prognosis were retrospectively analyzed. There were 9 males and 8 females patients with FⅤD, the median age was 36 (1-72 ) years. The median age of men was 39 (1-72)years, and the median age of women was 33 (8-56)years. There was no significant difference between them (=0.793). The median prothrombin time(PT) and activated partial thromboplastin time(APTT) values were 21.0(13.0-39.6) s and 54.6(38.2-121.2) s, and the median level of plasma FⅤ was 8.2% (0.9%-39%). Thirteen cases (13/17) were mild, 3 cases (3/17) were moderate, and only 1 case (1/17) was severe according to the FⅤ level. Five cases (5/17) had bleeding, including 3 cases with skin ecchymosis, 1 case with vaginal bleeding, and 1 case with gastrointestinal bleeding. According to the severity of bleeding, 1 case (1/17) had severe bleeding, and the other 4 cases (4/17) were mild bleeding. Patients with hereditary FⅤD either have or do not have bleeding symptoms, with prolonged PT and APTT and decreased plasma of FⅤ activity. There is no need for treatment for those without bleeding symptoms. Fresh frozen plasma is the first choice for patients who have bleeding, and the overall prognosis is good.
