Abstract Number: PB0970
Meeting: ISTH 2020 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Clinical
Background: Availability of factor replacement has dramatically improved care among hemophilia patients worldwide. However, the severity and frequency of bleeding is variable and the optimal management is complex with many challenging management decisions. In Malaysia, though the number of patients on prophylaxis therapy continue to rise, there are still some patients who are on-demand therapy.
Aims: To compare bleeding patterns and inhibitor development among hemophilia patients who received prophylaxis versus on-demand therapy.
Methods: Data was obtained from National Registry on inhibitor screening program for hemophilia patients in hospitals run by Ministry of Health (MOH), Malaysia. All eligible hemophilia A and B patients who consented to participate in the registry were included. Data were collected prospectively from 1st February 2015 until 1st February 2018. Data analysed with descriptive statistics.
Results: The study samples consisted of 210 hemophilia patients from the 16 participating MOH Centers. Median age was two (IQR 0.7;7.0) years old. Majority were diagnosed at infancy (n=99, 47.1%), followed by toddler and pre-school children with age between 2 to 5 years old (n=42, 20%). Malays has the highest number of hemophilia (n=104, 49.5%) and majority were diagnosed with Hemophilia A (n=194, 92.4%). Sixty four percent (n=134) of the study samples has moderate to severe disease however only 99 of cases (73.3%) were on prophylaxis. Overall median annual bleeding rates (ABR) was 2 (IQR 1.0;7.0). Mean ABR for moderate to severe hemophilia was lower among the prophylaxis arm (9.6%) with better Hemophilia Joint Health Score (HJHS), 12.6%. Six patients developed inhibitor during the study period and five were receiving on-demand therapy.
Conclusions: Prophylactic treatment was found to have better bleeding outcomes and joint health than on-demand therapy. Routine inhibitor screening is mandatory especially in patients receiving on-demand therapy.
To cite this abstract in AMA style:
Wilfred G, Selvaratnam V, Yap YY, Sathar J. Comparison of Bleeding Pattern and Inhibitor Development among Hemophilia Patients: Prophylactic versus On-Demand Treatment [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/comparison-of-bleeding-pattern-and-inhibitor-development-among-hemophilia-patients-prophylactic-versus-on-demand-treatment/. Accessed September 24, 2023.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/comparison-of-bleeding-pattern-and-inhibitor-development-among-hemophilia-patients-prophylactic-versus-on-demand-treatment/