Related Experiment Video
Updated: Nov 3, 2025
Investigating von Willebrand Factor Pathophysiology Using a Flow Chamber Model of von Willebrand Factor-platelet String Formation
Published on: August 14, 2017
Von Willebrand disease combined with coagulation defects in Iran
Omid Seidizadeh1, Minoo Ahmadinejad2, Sanaz Homayoun2
1"Angelo Bianchi Bonomi" Haemophilia and Thrombosis Centre, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and "Luigi Villa" Foundation, Milan, Italy.
Insights
Von Willebrand disease (VWD) combined with other coagulation defects occurs in 19.2% of Iranian VWD cases. These combined defects can cause more severe bleeding symptoms, requiring careful consideration for effective treatment.
Area of Science:
- Hematology
- Genetics
- Clinical Medicine
Background:
- Von Willebrand disease (VWD) is the most prevalent inherited bleeding disorder.
- Co-occurrence of VWD with other coagulation defects is rare but clinically significant.
- Limited data exists on the prevalence and characteristics of combined VWD and coagulation defects in Iran.
Purpose of the Study:
- To determine the clinical and laboratory features of VWD combined with other coagulation defects.
- To evaluate the prevalence of this combined condition in the Iranian population.
- To compare the clinical presentation of patients with combined defects versus VWD-only.
Main Methods:
- Retrospective analysis of 3,120 suspected VWD cases.
- Confirmation of VWD diagnosis and collection of clinical/laboratory data, including bleeding scores (BS).
- Subgroup analysis of 25 patients with dual/triple VWD and coagulation defects, compared to VWD-only and healthy controls.
Main Results:
- VWD was diagnosed in 130 patients; 25 had combined defects (FXII, FXI, FIX, FVII, FV, lupus anticoagulant).
- No significant differences in VWF laboratory measurements between combined and VWD-only groups.
- Combined defect patients exhibited higher median bleeding scores (4) compared to VWD-only (3) and controls (1).
Conclusions:
- The prevalence of combined coagulation defects in VWD patients in Iran is 19.2%.
- Co-occurrence of VWD with clotting factor deficiencies can result in more severe bleeding phenotypes.
- Consideration of combined defects is crucial for VWD patients with severe or refractory bleeding for optimal management.
Background:
Although Von Willebrand disease (VWD) is the most common inherited bleeding disorder, few cases of VWD combined with coagulation defects have been reported. This study sought to determine the clinical and laboratory features of VWD combined with other coagulation defects and to evaluate the prevalence of this combination in Iran.
Material And Methods:
A total of 3,120 cases were evaluated to confirm a suspected diagnosis of VWD. Clinical and laboratory phenotypes, including bleeding scores (BS), were also obtained.
Results:
A diagnosis of VWD was established for 130 patients. Following their further characterisation, a subgroup of 25 patients with a dual or triple combination of VWD with coagulation defects (FXII, FXI, FIX, FVII, FV, and lupus anticoagulant) was identified. Their laboratory and clinical data were compared with those of healthy controls (n=25) and VWD-only patients (n=25). No differences were observed for VWF-related laboratory measurements between the combined deficient cases and those with VWD only, results being expectedly lower than in healthy controls. The median BS of combined patients was 4, higher than for VWD-only and control groups (median BS 3 and 1; p=0.55 and p<0.001, respectively).
Discussion:
The prevalence of combined coagulation defects was 19.2% among all the VWD cases. The co-occurrence of VWD with clotting factor deficiencies may lead to more severe clinical presentations. To ensure adequate treatment, combined defects should be considered in VWD patients presenting with a more severe bleeding phenotype than expected or with a poor response to treatment.
