Background: Von Willebrand’s disease (VWD) is a common inherited bleeding disorder.

Aims: Our study aims to access the information regarding clinical presentation and frequency of types of Von Willebrand Disease in Pakistan.

Methods: A cross sectional study was carried out in Sir GangaRam Hospital Lahore from Dec 2017 to Nov 2018. 50 patients were included in the study. The present study was carried out to find the frequency of VWD, its types, and clinical features of the disease. Informed consent was obtained.
A detailed personal and family history of bleeding manifestations including onset, signs, and symptoms were noted. Complete Blood Count (CBC), bleeding time (BT), Prothrombin time (PT), Activated Partial Thromboplastin Time (APTT), fibrinogen and blood grouping were the laboratory tests performed who met inclusion criteria.

Results: 23 (46%) patients were male and 27 (54%) were female. Type3 VWD (92.12%) was found to be the commonest type. Two (5.92%) cases of type-2 VWD and only one (1.96%) case of type-1 VWD were identified. Easy bruising was the most commonly observed clinical presentation, 23 (46.13%) patients, followed by menorrhagia ( 20.40%), epistaxis (11.68%), bleeding after surgery (9.68%), bleeding after circumcision (6.45%), umbilical cord bleeding (4.92). Consanguineous marriages were reported in parents of (84.6%) patients.

Conclusions: Type-3 VWD was found to be the commonest type which can be attributed to the fact that type-3 VWD is transmitted through autosomal recessive pattern of inheritance and consanguineous marriages are highly practiced in our society leading to high frequency of this form of VWD. Easy bruising, menorrhagia and epistaxis were concluded to be the most common clinical presentation.

« Back to ISTH 2020 Congress

ISTH Congress Abstracts - https://abstracts.isth.org/abstract/clinical-presentation-of-von-willebrand-disease-in-a-developing-country-a-cross-sectional-study/