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Int J Lab Hematol. 2018 May 13. doi: 10.1111/ijlh.12850. [Epub ahead of print]

Bleeding score in type 1 von Willebrand disease patients using the condensed MCMDM-1 vWD validated questionnaire.

Author information

1
Sultan Qaboos University Hospital, Muscat, Oman.
2
College of Medicine & Health Sciences, Sultan Qaboos University, Muscat, Oman.

Abstract

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].

KEYWORDS:

BS; MCMDM-1; Questionnaire; bleeding score; vWD

PMID:
29754468
DOI:
10.1111/ijlh.12850

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