Clinical Spectrum and Laboratory Study of Von Willebrand Disease -Experience from Tertiary Care Hospital in Pakistan

  • Mohsin Hussain Armed Forces Institute of Pathology/National University of Medical Sciences (NUMS), Rawalpindi Pakistan
  • Asad Mahmood Armed Forces Institute of Pathology/National University of Medical Sciences (NUMS), Rawalpindi Pakistan
  • Rafia Mahmood Armed Forces Institute of Pathology/National University of Medical Sciences (NUMS), Rawalpindi Pakistan
  • Ayesha Khurshid Armed Forces Institute of Pathology/National University of Medical Sciences (NUMS), Rawalpindi Pakistan
  • Nabeela Khan Armed Forces Institute of Pathology/National University of Medical Sciences (NUMS), Rawalpindi Pakistan
  • Nozaif Sarwar Armed Forces Institute of Pathology/National University of Medical Sciences (NUMS), Rawalpindi Pakistan
Keywords: Bleeding time, Factor viii, Inherited, Von wille-brand disease, Von wille-brand antigen

Abstract

Objective: To determine the clinical features and laboratory parameters of patients of von Willebrand disease (vWD) in our population.

Study Design: Cross-sectional study.

Place and Duration of Study: Department of Hematology, Armed Forces Institute of Pathology (AFIP), Rawalpindi Pakistan, from Jan to Jul 2019.

Methodology: All patients newly diagnosed von Willebrand disease patients were assessed clinically. Complete blood counts, bleeding time, coagulation profile, von Willebrand antigen levels and FVIII levels were determined.

Results: A total of 66diagnosed patients of von Willebrand disease were included in the study. Out of these 15 (22.7%) were male while 51 (77.2%) were females. The most common clinical symptom was pallor, seen in 49 (74.2%) patients, followed by epistaxis in 36 (54.5%) and gum bleeding in 28 (42.4%) patients. Consanguineous marriages were found in 40 cases (60.6%) and family history was positive in 23 (34.8%). Patients mean vWF Ag level was4.29 ± 7.7 IU/dL while mean FVIII levels was 5.18 ± 7.8 1 U/mL.

Conclusion: Von Willebr and disease is among the most common inherited bleeding disorder which presents with a classical pattern of mucocutaneous/soft tissue bleed, with pallor epistaxis and gum bleed being the most common presenting complaints.

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Published
2022-05-31
How to Cite
Hussain, M., Mahmood, A., Mahmood, R., Khurshid, A., Khan, N., & Sarwar, N. (2022). Clinical Spectrum and Laboratory Study of Von Willebrand Disease -Experience from Tertiary Care Hospital in Pakistan. Pakistan Armed Forces Medical Journal, 72(SUPPL-2), S107-10. https://doi.org/10.51253/pafmj.v72iSUPPL-2.3101
Section
Original Articles