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Bleeding score in type 1 von Willebrand disease patients using the condensed MCMDM‐1 vWD validated questionnaire
Author(s) -
Pathare A.,
Al Hajri F.,
Al Omrani S.,
Al Obaidani N.,
Al Balushi B.,
Al Falahi K.
Publication year - 2018
Publication title -
international journal of laboratory hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.705
H-Index - 55
eISSN - 1751-553X
pISSN - 1751-5521
DOI - 10.1111/ijlh.12850
Subject(s) - interquartile range , medicine , von willebrand disease , von willebrand factor , gastroenterology , cohort , platelet
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].