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

1Department of Haematology, Ampang Hospital, Ministry of Health Malaysia, Kuala Lumpur, Kuala Lumpur, Malaysia, 2Department of Haematology, Ampang Hospital, Ministry of Health Malaysia, Selangor, Malaysia, 3Clinical Research Centre, Ampang Hospital, Ministry of Health Malaysia, Ampang, Selangor, Malaysia, 4Department of Haematology, Ampang Hospital, Ministry of Health Malaysia, Ampang Jaya, Selangor, Malaysia

Abstract Number: PB0188

Meeting: ISTH 2022 Congress

Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Basic

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.

Table 1

Type & Severity of Haemophilia

Image 1

Number of Bleeding Episodes by Type of Haemophilia

To cite this abstract in AMA style:

Nasution S, Kunju T, Edmund S, Selvaratnam V, Sathar J. World Bleeding Disorders Registry (WBDR): Congenital Haemophilia, Ampang Hospital Experience [abstract]. https://abstracts.isth.org/abstract/world-bleeding-disorders-registry-wbdr-congenital-haemophilia-ampang-hospital-experience/. Accessed September 27, 2023.

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