1.Demographics and outcome of patients with congenital haemophilia in Sarawak, Malaysia
Andy Tang Sing Ong ; Wong Qi Ying ; Tan Yee Yen ; Chieng Chae Hee ; Ko Ching Tiong ; Ong Gek Bee ; Chew Lee Ping
The Medical Journal of Malaysia 2021;76(1):51-55
Introduction: Sarawak has a population that is
geographically and characteristically widely varied. This
study aimed to determine the demographic profile of
patients in Sarawak, Malaysia.
Materials and Methods – A cross-sectional study was
conducted in 2019 at four major haemophilia treatment
centres in Kuching, Sibu, Bintulu and Miri Hospitals,
Sarawak. Demographic and clinical data were collected with
consents from patients.
Results and Discussion: Ninety-six haemophilia patients
were identified - 79(82.3%) haemophilia A(HA) and 17(17.7%)
haemophilia B(HB). Severe haemophilia patients were noted
in 45.6% (36/79) of HA and 64.7% (11/17) of HB. In all 44.3%
of the HA and 52.9% of the HB population had no identifiable
family history of haemophilia. Two-thirds of the patients with
severe HA were on prophylaxis [24/36 (66.7%)] and only onethird [4/11 (36.4%)] in severe HB. Inhibitors developed in 9/79
(11.4%) of the HA population [3/79 (3.8%) high responders].
The median inhibitor titre was not significantly different
between the different treatment groups – on demand versus
prophylaxis (1.0BU versus 2.0BU; z statistic -1.043, p-value
0.297, Mann-Whitney test). None of the patients developed
inhibitory alloantibodies to factor IX. Four HA patients (5.1%)
underwent immune tolerance induction where one case had
a successful outcome. Three severe HA patients received
emicizumab prophylaxis and showed remarkable reduction
in bleeding events with no thromboembolic events being
reported. One female moderate HA patient received
PEGylated recombinant anti-haemophilic factor. Eleven
patients underwent radiosynovectomy. One mild HB patient
succumbed to traumatic intracranial bleeding. Our data
reported a prevalence (per 100,000 males) of 5.40 cases for
all severities of HA, 2.46 cases for severe HA; 1.16 cases for
all severities of HB, and 0.75 cases for severe HB. The
overall incidence of HA and HB was 1 in 11,500 and 1 in
46,000, respectively.
Conclusion: This study outlines the Sarawakian haemophilia
landscape and offers objective standards for forward
planning. Shared responsibilities among all parties are of
utmost importance to improve the care of our haemophilia
population.