Your browser doesn't support javascript.
loading
Congenital factor X deficiency: a retrospective analysis of 11 cases / 中华血液学杂志
Chinese Journal of Hematology ; (12): 26-30, 2022.
Article in Chinese | WPRIM | ID: wpr-929525
ABSTRACT

Objective:

To analyze the clinical characteristics, laboratory examination, diagnosis, treatment, and outcome of hereditary factor Ⅹ (FⅩ) deficiency.

Methods:

Clinical data of 11 patients with congenital FⅩ deficiency were retrospectively analyzed from July 2009 to February 2021.

Results:

There were 3 males and 8 females. Median age was 39 (5-55) years. The media duration of follow-up was 81.67 (1.87-142.73) months. Of the 11 patients, 10 had bleeding symptoms, 7 had ecchymosis or hemorrhage after skin bump, 7 had nosebleed, 6 had gingival hemorrhage, and 1 had muscle hematoma. Among the female patients, 6 had menorrhagia and 1 experienced bleeding after vaginal delivery. Family history of FⅩ deficiency was found in one case. Eight patients had a history of surgery, and four had postoperative bleeding. Laboratory findings were characterized by significantly prolonged activated partial thromboplastin time, prothrombin time, and decreased FⅩ activity (FⅩ∶C) . Four cases underwent gene mutation analysis and five new mutations were found. Four cases were treated with prothrombin complex concentrates (PCC) and seven cases with fresh frozen plasma (FFP) . One female patient had significantly reduced menstrual volume after PCC prophylactic therapy. One patient received FFP for prophylactic infusion with no bleeding during and after the operation.

Conclusion:

Most patients with congenital FⅩ deficiency had bleeding symptoms and there was no significant correlation between severity of bleeding symptoms and FⅩ∶C. Prophylaxis should be applied in patients with severe bleeding tendencies. Gene mutation test is significant for screening, diagnosis, and prognosis prediction of congenital FX deficiency.
Subject(s)

Full text: Available Index: WPRIM (Western Pacific) Main subject: Plasma / Blood Coagulation Tests / Blood Coagulation Factors / Retrospective Studies / Factor X Deficiency / Hemorrhage Type of study: Observational study / Prognostic study Limits: Adolescent / Adult / Child / Child, preschool / Female / Humans / Male Language: Chinese Journal: Chinese Journal of Hematology Year: 2022 Type: Article

Similar

MEDLINE

...
LILACS

LIS

Full text: Available Index: WPRIM (Western Pacific) Main subject: Plasma / Blood Coagulation Tests / Blood Coagulation Factors / Retrospective Studies / Factor X Deficiency / Hemorrhage Type of study: Observational study / Prognostic study Limits: Adolescent / Adult / Child / Child, preschool / Female / Humans / Male Language: Chinese Journal: Chinese Journal of Hematology Year: 2022 Type: Article