PB0647 - Life expectancy and mortality of patients with haemophilia A and haemophilia B in Australia

Monday, June 26, 2023

18:30 – 19:30 ET

Room: Exhibition

Co-Author(s)

Sumit PARIKH, PhD

ABDR Senior Research Fellow
Australian Haemophilia Centre Directors’ Organisation, Melbourne, VIC, Australia
Melbourne, Victoria, Australia

Co-Author(s)

CT

Chee Wee TAN

Clinical Director
SA Pathology, Adelaide, SA, Australia
Adelaide, South Australia, Australia
SB

Simon BROWN

Clinical Director
Queensland Children Hospital, Brisbane, QLD, Australia
Brisbane, Queensland, Australia
CB

Chris BARNES

Clinical Director
Royal Children Hospital, Melbourne, VIC, Australia
Melbourne, Victoria, Australia
SP

Stephanie P’NG

Haematologist
Fiona Stanley Hospital, Perth, WA, Australia
Perth, Western Australia, Australia
TC

Tina CARTER

Haematologist
Perth Children Hospital, Perth, WA, Australia
Perth, Western Australia, Australia

Presenting Author(s)

HT

Huyen Tran

Professor
Australian Centre for Blood Diseases, Monash University
Melbourne, Victoria, Australia

Background: Patients with severe haemophilia, characterised by baseline factor levels of less than 1%, represent approximately one- third of total haemophilia A (HA) and one- fifth of total haemophilia B (HB) population in Australia. Prescribing prophylaxis with regular infusions of the missing factor concentrate for such patients or more recently with non-factor replacement such as emcizumab is a well-established standard of care. However life expectancy and mortality remains unknown in this group.

Aims: To assess impact of severity and prophylaxis on life expectancy, mortality in patients with HA and HB in Australia, develop survival models based on all-cause mortality and examine probability of survival by severity and treatment regimen.

Methods: A national, retrospective study was conducted using data extracted from the Australian Bleeding Disorder Registry (ABDR). Data was obtained on severity, age, treatment regimen and mortality in patients with HA and HB. Patient demographics and results presented are based on ABDR data up until October 2022.

Results: There are 2874 HA and 654 HB patients in the ABDR. All-cause mortality in HA accounted for 13.5%, 11.5% and 7.6% in severe, moderate and mild patients respectively. In HB, that was 11.7%, 5.4% and 9.8% in severe, moderate and mild patients respectively. Estimated median life expectancy is 79 years for HA and 82 years for HB patients, which is slightly lower than median life expectancy of general Australian population (83 years). Severity significantly impacts estimated median life expectancy, reducing to 72 years in severe HA and 68 years in severe HB. Prophylaxis favourably improved estimated median life expectancy to 78 years in severe HA, whereas reduced to 55 years in non-prophylaxis group.

Conclusion(s): This study provides valuable insights of survival in patients with haemophilia in Australia.