Abstract Number: PB1202
Meeting: ISTH 2020 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Rare Bleeding Disorders
Background: Sex differences may influence bleeding phenotype, diagnosis and management of patients with autosomal inherited bleeding disorders.
Aims: Therefore, we investigated sex differences in a large cohort of well-defined patients with autosomal inherited bleeding disorders (i.e. von Willebrand disease (VWD) and rare bleeding disorders (RBDs)).
Methods: We included all patients with VWD and RBDs in the Netherlands from two nationwide cross-sectional studies on VWD (WiN study) and RBDs (RBiN study). In both studies, the bleeding score(BS) was obtained, patients filled in an extensive questionnaire and blood was drawn for central measurement of coagulation factor levels.
Results: We included 1030 patients (834 VWD; 196 RBD patients), the baseline characteristics are shown in Table 1. The mean age of diagnosis was 15.7 ±(SD: 20.3) years in men, and 21.1 ±20.9 years in women (p< 0.001). Women were more often referred for bleeding than men: 276 (46.1%) vs 133 (34.3%) (p< 0.001, Figure 1). In patients referred for bleeding, mean age of first bleeding was not different between men (9.1 ±13.0 years) and women (10.8 ±11.1 years, p=0.229). However, mean age of diagnosis was much lower in men referred for bleeding than in women: 18.0 ±19.5 years vs 25.4 ±19.3 years (p< 0.001). Thus, the diagnostic delay after the first bleeding episode was much longer in women (14.0 ±16.6 years) than in men (8.1 ±17.0 years, p< 0.001). Total BS was lower in men than in women (9.7 ±6.9 vs 11.6 ±7.3, p< 0.001). However, after deduction of sex specific bleeding, BS seemed higher in men (9.7 ±6.9) compared to women (8.8 ±6.1, p=0.053). In women aging 12 years or older, 63.9% had undergone treatment because of menorrhagia or postpartum hemorrhage.
Conclusions: In this large combined nationwide study we found important differences in bleeding phenotype, diagnostic delay, and management of males and females with autosomal inherited bleeding disorders.
Type 1 VWD | Type 2 VWD | Type 3 VWD | RBDs1 | |||||
Male n=173 (35%) |
Female n=321 (65%) |
Male n=148 (47%) |
Female n=164 (53%) |
Male n=14 (50%) |
Female n=14 (50%) |
Male n=70 (36%) |
Female n=126 (64%) |
|
Age inclusion | 33.9 ±20.9* | 43.1 ±17.9* | 36.4 ±21.6 | 38.9 ±20.9 | 30.7 ±23.0 | 28.7 ±21.2 | ||
VWF antigen | 0.35 ±0.24 | 0.40 ±0.22 | 0.27 ±0.16 | 0.27 ±0.15 | 0.01 ±0.01 | 0.01 ±0.01 | ||
VWF activity2 | 0.44 ±0.34 | 0.49 ±0.32 | 0.14 ±0.24 | 0.12 ±0.12 | 0.00 ±0.01 | 0.00 ±0.00 | ||
Coagulation factor activity3 | 0.23 ±0.28 | 0.24 ±0.30 | ||||||
Fibrinogen activity4 | 0.46 ±0.26 | 0.70 ±0.40 | ||||||
Euglobulin clot lysis time ratio5 | 11.0 ±2.4 | 8.1 ±2.8 | ||||||
Bleeding score | 8.1 ±6.3* | 10.7 ±7.0* | 10.7 ±6.5* | 13.0 ±7.7* | 18.9 ±6.0 | 19.9 ±8.4 | 9.9 ±7.9 | 11.4 ±6.7 |
Data presented as mean ±standard deviation or frequency (percentage). *p<0.05 between men and women.1152 patients with rare coagulation factor deficiencies and 44 with disorders of fibrinolytic system. 2Measured with the monoclonal antibody assay.3Measured in patients with a rare coagulation factor deficiency.4Measured in patients with a fibrinogen deficiency.5Measured in patients with PAI-1 deficiency and hyperfibrinolysis |
[Table 1. Patient characteristics]
[Figure 1. Reason for referral in patients with autosomal inherited bleeding disorders]
To cite this abstract in AMA style:
Atiq F, Saes JL, Meijer K, Cnossen MH, Schutgens REG, Laros-van Gorkom BAP, Peters M, Nieuwenhuizen L, van Galen KPM, de Meris J, van der Bom JG, van der Meer FJM, Fijnvandraat K, Kruis IC, van Heerde WL, Eikenboom J, Schols SEM, Leebeek FWG, WiN and RBIN Study Groups . Important Sex Differences in Patients with Autosomal Inherited Bleeding Disorders [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/important-sex-differences-in-patients-with-autosomal-inherited-bleeding-disorders/. Accessed March 21, 2024.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/important-sex-differences-in-patients-with-autosomal-inherited-bleeding-disorders/