PB0188 - World Bleeding Disorders Registry (WBDR): Congenital Haemophilia, Ampang Hospital Experience

Sunday, July 10, 2022

6:30 PM – 7:30 PM

Location: Exhibition

Presentation Format: In-Person

Presenting Author(s)

Syazarin Fidhora Nasution, MBChB

Medical Resident
Department of Haematology, Ampang Hospital, Ministry of Health Malaysia
Kuala Lumpur, Kuala Lumpur, Malaysia

Co-Author(s)

Tanusha Kunju, MD

Medical Officer
Department of Haematology, Ampang Hospital, Ministry of Health Malaysia, Selangor, Malaysia
SE

Syed Carlo Edmund

Research Officer
Clinical Research Centre, Ampang Hospital, Ministry of Health Malaysia
Ampang, Selangor, Malaysia
VEENA SELVARATNAM

Clinical Haematologist
Ampang Hospital
Ampang, Selangor, Malaysia
JS

Jameela Sathar

Consultant Haematologist
Department of Hematology, Hospital Ampang, Ampang, Selangor, Malaysia
Ampang, Selangor, Malaysia

Background: Ampang Hospital (AH) is a Haemophilia Comprehensive Care Centre (CCCH) in the central region of Malaysia and national referral centre for bleeding disorders. Our centre has one of the highest numbers of patients with haemophilia (PWH) in Southeast Asia. AH uses the WBDR; a web-based system to gather data of PWH.

Aims: To describe the epidemiology of congenital haemophilia, evaluate & improve the quality of care of PWH in AH.

Methods: A total of 232 PWH which represent 16% of total PWH in Malaysia were registered into the WBDR from April 1, 2019 until May 20, 2020. Clinical records were derived retrospectively from electronic Hospital Information System (eHIS), 2006-2020. Inclusion criteria; PWH in AH with congenital Haemophilia A/B. Acquired haemophilia and other congenital bleeding disorders were excluded.

Results: Most PWH were diagnosed at 0-12 months of age. The median age for PWH was 33 years old. Severe haemophilia represents the majority of cases (75%) followed by mild 13.8% and moderate 11.2%. 6% of 188 haemophilia A (HA) & 1.3% haemophilia B (HB) developed inhibitors. All 17 PWH with inhibitors being severe haemophilia; 9 of them needing bypassing agents (4 on FEIBA and 5 on NovoSeven). 76% non-inhibitor patients with HA require factor VIII concentrates (75.5% on prophylaxis). As for HB, 77.3% require factor IX concentrates (59.1% on prophylaxis). Haemarthrosis was the most common breakthrough bleed. Bleeding rate was the highest in the severe group (78.7%), followed by moderate & mild group (16.7%). Mean annual bleeding rate was 2.9 in prophylaxis and 3.8 in on-demand group.

Conclusion(s): Mean annual bleeding rate was higher in on-demand group. Bleeding rate was high in the severe group despite the introduction of prophylaxis regime. Prophylaxis infusion should be started earlier amongst severe PWH. The introduction of myWBDR mobile application may optimise treatment outcome & improve PWH quality of life.