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Bleeding score in type 1 von Willebrand disease patients using the condensed MCMDM-1 vWD validated questionnaire
A Pathare1, F Al Hajri2, S Al Omrani2
1Sultan Qaboos University Hospital, Muscat, Oman.
Insights
The bleeding score (BS) effectively measures bleeding severity in type 1 von Willebrand disease (VWD) patients. However, this score did not significantly correlate with laboratory markers in Omani patients.
Area of Science:
- Hematology
- Clinical Diagnostics
- Genetic Disorders
Background:
- Validated bleeding assessment tools are crucial for evaluating bleeding symptom severity.
- Type 1 von Willebrand disease (VWD) is a common inherited bleeding disorder.
Purpose of the Study:
- To administer the condensed molecular and clinical markers for the diagnosis and management of type 1 von Willebrand disease (MCMDM-1 vWD) questionnaire to Omani patients.
- To correlate the bleeding score (BS) with laboratory parameters in type 1 VWD patients.
Main Methods:
- The MCMDM-1 vWD questionnaire was administered to Omani type 1 VWD patients and controls.
- Bleeding scores (BS) were calculated based on reported bleeding symptoms.
- Spearman's correlation was used to assess the relationship between BS and laboratory parameters.
Main Results:
- Oral cavity bleeding was the most common symptom (63%).
- The median BS was 5, with no significant difference between genders.
- BS showed weak to moderate correlations with FVIII:C, VWF:RCo, VWF:Ag, and VWF:CB levels.
Conclusions:
- The bleeding score (BS) reflects bleeding severity in type 1 VWD patients.
- The BS did not show a statistically significant correlation with laboratory parameters in this cohort.
Background:
Assessment of the severity of bleeding symptom has led to the evolution of bleeding assessment tools which are now validated.
Aims:
To administer the condensed molecular and clinical markers for the diagnosis and management of type 1 von Willebrand disease VWD (MCMDM-1 vWD) questionnaire to the Omani type 1 vWD patients and correlate it with the laboratory parameters.
Methods:
Patients and controls were personally interviewed and the condensed MCMDM-1 vWD questionnaire administered by a single investigator. Bleeding score (BS) was calculated, based on the presence or absence of the bleeding symptoms according to a standard validated questionnaire in both the patients and the controls.
Results:
The median age of the patient cohort was 27 (range, 7-49) years with 60.87% of females. The median time to administer condensed MCMDM-1 BS questionnaire was 11 minutes (interquartile range-IQR;7,16). Overall, bleeding from the oral cavity was the most predominant symptom (63%). The median BS was 5 (IQR;1,8) although individual scores ranged between 0 and 29. However, there was no statistically significant difference in BS between genders (males: median 4; IQR 1,6 and females: median 5, IQR 1,10) (P > .05, Kruskal-Wallis test) The Spearman's correlation value of BS was weak with FVIII:C levels and von Willebrand Ristocetin co-factor activity; very weak with von Willebrand Antigen level, and moderate with vonWillebrand Collagen Binding activity being -0.29, -0.28, -0.14 and -0.43, respectively.
Conclusion:
The BS reflects the severity of bleeding among the vWD patients. Although the BS was abnormal, it did not correlate significantly with the surrogate laboratory parameters [P > .05].
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