Abstract Number: PB1197
Meeting: ISTH 2020 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Rare Bleeding Disorders
Background: Factor VII (FVII) deficiency is a rare inherited bleeding disorder. There is poor correlation between FVII level and bleeding phenotype. There is little published information about the relationship with bleeding in women.
Aims: To describe diagnosis, type of bleeding, treatment and outcome of women with FVII deficiency.
Methods: Retrospective cohort study. Consecutive women from a university hospital, age >18y.o. with FVII deficiency (2016-2019) were included (FVII< 50%). Prothrombin Time (PT) was performed by a rabbit brain thromboplastin (PTFibrinogenHS+) and FVII dosage by one stage clotting assay with human recombinant thromboplastin (RecombiPlasTin2G) at three dilutions in a ACL TOP. Inhibitor presence was ruled out by PT and FVII mixing studies.
Reasons for determining FVII were evaluated. Spontaneous, peri-surgical bleeding and bleeding related to the peri-partum were recorded. Bleeding: classified according to ISTH guidelines. Statistic: basic descriptive analysis in Stata13.
Results: Twenty-eight women with FVII deficiency were included. Basic characteristics of the population in table 1. In 54% the diagnosis was suspected by a prolongation (frequently slight) of PT in patients without a history of bleeding.No FVII deficiency was acquired. Only 17% were diagnosed by bleeding.
Most patients (62%) had a PT greater than 60%. No patient had FVII < 10% at diagnosis and only 32% FVII< 30%.
Eight (29%) of the patients had a history of abnormal uterine bleeding, but none from menarche. Two patients had an alternative gynecological cause of bleeding.
Of the total population, only two patients had a history of major bleeding.
Table 2 shows the results of patients facing hemostatic challenges.
Conclusions: In our cohort of mild FVII deficiency we observed a low rate of bleeding complications. Most of the patients had no history of menorrhagia and no complications related to delivers were observed. We did not have patients with severe deficiency, being this a study limitation.
| Patients included, n | 28 |
| Age in years, median (IQR) | 35 (25-45) |
| Factor VII in U/dL, median (IQR) | 37 (28.5-44.5) |
| PT activity in %, median (IQR) | 64 (57.5-69) |
| Reason for determination of FVII | |
| PT alteration in routine controls without bleeding, n(%) | 15 (54) |
| Pre-surgery study, n (%) | 7 (25) |
| Bleeding, n (%) | 5 (18) |
| Family history of FVII deficiency, n (%) | 1 (3) |
[Table 1: Baseline characteristics of the population]
| Major surgery, events (patients) | 13 (12) | Minor surgery, events (patients) | 12 (12) | Deliveries, events (patients) | 11 (10) |
| Preventive treatment, n (%) | 8 (61.5) | Preventive treatment, n (%) | 2 (17) | Preventive treatment, n (%) | 0 |
| Preventive treatment: Recombinant factor VII / fresh frozen plasma, n / n | 4/4 | Preventive treatment: Recombinant factor VII / fresh frozen plasma, n / n | 1/1 | Caesarean section, n (%) | 8 (73) |
| Bleeding, n | 0 | Bleeding, n | 1 | Bleeding, n | 2 (18) |
[Table2: Hemostatic Challenges]
To cite this abstract in AMA style:
Chuliber F, Martinuzzo M, Privitera V, Lopez M, Viñuales E, Barrera L, Mezzarobba D, Fotti Berdasco ME, Villagra Iturre M, Trulls M, Penchasky D, Arbelbide J. Inherited Factor VII Deficiency in Women: Low Rate of Bleeding in Non-severe Disease [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/inherited-factor-vii-deficiency-in-women-low-rate-of-bleeding-in-non-severe-disease/. Accessed October 1, 2023.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/inherited-factor-vii-deficiency-in-women-low-rate-of-bleeding-in-non-severe-disease/