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. 2022 Jul 22;28(6):950–961. doi: 10.1111/hae.14638

TABLE 1.

Summary of differences in standards of care and inhibitor management between Western and non‐Western countries as reported by the authors

UK Italy Turkey India Egypt
Inhibitor diagnosis and testing
  • Testing up to four times a year

  • Inhibitor screening is part of a comprehensive haemophilia programme allowing routine screening and timely detection of inhibitors

  • PUPs are evaluated every five EDs up to 50 EDs

  • Inhibitor screening and testing is performed routinely in most of the centres every five EDs up to 50 EDs

  • All centres have expert laboratory technicians following the launch of the national inhibitor screening programme 15 years ago

  • Testing performed opportunistically, only in patients who do not respond to treatment

  • Mass inhibitor testing is sometimes carried out with support from industry or in community‐based camps

  • Laboratories lack centralisation resulting in unreliable testing and reading of results

  • City‐to‐city variations and inequities in resource allocation

  • Routine inhibitor screening is performed biannually

Morbidity/mortality associated with inhibitors
  • Patients used to suffer from poor QoL, which usually translated into high absenteeism from work or school; nevertheless, the availability of extended half‐life products and non‐replacement therapies has remarkably helped improve the QoL of patients

  • Patients develop arthropathies and have poorer QoL than patients without inhibitors

  • Mortality in patients with inhibitors is low because of prophylaxis treatment

  • The prevalence of joint damage and pseudo‐tumours is high because of untreated bleeds

  • Mortality due to cerebral bleeds is anecdotally elevated

  • Patients experience severe complications, which are usually less common in those without inhibitors

ITI adoption
  • With the recent introduction of alternative non‐factor replacement therapies in Europe, physicians and patients are opting for less complex and demanding prophylactic therapies with improved regimens to avoid the use of ITI

  • Only low‐dose ITI with plasma‐derived FVIII (50 IU/kg thrice weekly for 6 months, and a decreasing dose for another 6 months) and sometimes no ITI at all. High dose ITI is only available through clinical trials

  • ITI is not done adequately as it requires financial resources, constant availability of factor, and patient commitment, all of which are lacking

BPA use
  • The preventive treatment of inhibitor‐related bleeds is shifting towards the use of newer agents with improved mode and frequency of administration such as non‐factor‐based agents

  • Longstanding successful experience with BPAs for the management of bleeding peri‐ and post‐operatively

  • BPAs are expensive and not widely available, so physicians try to delay elective surgery as much as possible to avoid their use; real‐world data on experience with these agents in emergency settings are scarce

  • Patients receive either aPCC or rFVIIa as pre‐ and post‐surgical interventions based on the WFH recommendations

Use of non‐factor replacement agents
  • Most of the European patients with inhibitors have now been switched to emicizumab, following the favourable outcomes achieved in emicizumab's clinical programme and in the real world

  • Experience with emicizumab is limited to eight paediatric patients who were enrolled in HAVEN 2 for 3 years

  • The Ministry of Health approved it at the end of 2019; however, it is still not reimbursed and thus not widely accessible

  • Emicizumab is available through patient access programs only

  • It is unlikely to be licensed soon given its high cost, especially with the anticipated shift in focus of the government amidst the COVID‐19 pandemic

  • Physicians have been using emicizumab for 14 months, with very good results so far

aPCC, activated prothrombin complex concentrate; BPAs, bypassing agents; ED, exposure day; FVIII, factor VIII; ITI, immune tolerance induction; PUP, previously untreated patient; QoL, quality of life; rFVIIa; recombinant factor VIIa; WFH, World Federation of Hemophilia.