Treatment of female patients with inherited bleeding disorders in reproductive age: a single centre study from northern Pakistan

Treatment of Female Patients with Inherited Bleeding Disorders

Abstract

ABSTRACT

Introduction: Inherited bleedings disorders (IBD) in women are mismanaged in Pakistan due to lack of standardized treatment options. The most common is Von Willibrand Disease (VWD) followed by rare bleeding disorders (RBD). Heavy menstrual bleeding (HMB) and postpartum hemorrhage (PPH) are the main clinical manifestations causing lot of morbidity and mortality.

Objective: To document current treatment practices in female patients in reproductive age with IBD.
Study Design: A retrospective study with analysis of patients' records from Hemophilia Treatment Centre (HTC) Rawalpindi.

Duration: June 2017 – June 2022

Place: HTC, Haemophilia Patient Welfare Society, Rawalpindi.

Patients and Methods: Female patients with IBD aged between 11-45 years were included in study. VWD, RBD like deficiencies of factors (I, II, V, VII, X, XI, XIII) and Platelet Function Disorders e.g. Glanzman Thrombesthenia (GTT) and Bernard Soulier Syndrome (BSS) as well as Hemophilia carriers. HMB and PPH were documented along with treatment given.

Results: Total 67 patients were included. Age of Menarche was between 11 to 15 years. 89.4% patients with VWD, 60% with RBD and 100% with platelet function defects had HMB. Antifibrinolytic agent was the most common treatment followed by FFP infusion (70 – 86%). Factor replacement was done in 57.5% and OCPs were used in 50% patients. Eleven patients conceived, 10 had full term deliveries and 5 had PPH. They received antifibrinolytics, FFP and factor concentrates.

Conclusion: In our centre, antifibrinolytics were the most common treatment followed by FFPs, OCPs, hormones and factor concentrates.