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New Inhibitors in the Ageing Population : A Retrospective, Observational, Cohort Study of New Inhibitors in Older People with Hemophilia

Astermark, Jan LU ; Ay, Cihan ; Carvalho, Manuela ; D'Oiron, Roseline ; Moerloose, Philippe De ; Dolan, Gerard ; Fontana, Pierre ; Hermans, Cedric ; Holme, Pål Andre and Katsarou, Olga , et al. (2022) In Thrombosis and Haemostasis 122(6). p.905-912
Abstract

Introduction A second peak of inhibitors has been reported in patients with severe hemophilia A (HA) aged >50 years in the United Kingdom. The reason for this suggested breakdown of tolerance in the aging population is unclear, as is the potential impact of regular exposure to the deficient factor by prophylaxis at higher age. No data on hemophilia B (HB) have ever been reported. Aim The ADVANCE Working Group investigated the incidence of late-onset inhibitors and the use of prophylaxis in patients with HA and HB aged ≥40 years. Methods A retrospective, observational, cohort, survey-based study of all patients aged ≥40 years with HA or HB treated at an ADVANCE hemophilia treatment center. Results Information on 3,095 people aged ≥40... (More)

Introduction A second peak of inhibitors has been reported in patients with severe hemophilia A (HA) aged >50 years in the United Kingdom. The reason for this suggested breakdown of tolerance in the aging population is unclear, as is the potential impact of regular exposure to the deficient factor by prophylaxis at higher age. No data on hemophilia B (HB) have ever been reported. Aim The ADVANCE Working Group investigated the incidence of late-onset inhibitors and the use of prophylaxis in patients with HA and HB aged ≥40 years. Methods A retrospective, observational, cohort, survey-based study of all patients aged ≥40 years with HA or HB treated at an ADVANCE hemophilia treatment center. Results Information on 3,095 people aged ≥40 years with HA or HB was collected. Of the 2,562 patients with severe HA, the majority (73% across all age groups) received prophylaxis. In patients with severe HA, the inhibitor incidence per 1,000 treatment years was 2.37 (age 40-49), 1.25 (age 50-59), and 1.45 (age 60 +). Overall, the inhibitor incidence was greatest in those with moderate HA (5.77 [age 40-49], 6.59 [age 50-59], and 4.69 [age 60 + ]) and the majority of inhibitor cases were preceded by a potential immune system challenge. No inhibitors in patients with HB were reported. Conclusion Our data do not identify a second peak of inhibitor development in older patients with hemophilia. Prophylaxis may be beneficial in older patients with severe, and possibly moderate HA, to retain a tolerant state at a higher age.

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organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
aging, hemophilia A/B, inhibitors, prophylaxis
in
Thrombosis and Haemostasis
volume
122
issue
6
pages
905 - 912
publisher
Georg Thieme Verlag
external identifiers
  • pmid:34507368
  • scopus:85119113861
ISSN
0340-6245
DOI
10.1055/a-1642-4067
language
English
LU publication?
yes
additional info
Publisher Copyright: © 2021 Georg Thieme Verlag. All rights reserved.
id
9b6248f5-1299-4f93-89f9-7b79a4b4b13d
date added to LUP
2021-12-03 15:22:52
date last changed
2025-04-04 14:55:45
@article{9b6248f5-1299-4f93-89f9-7b79a4b4b13d,
  abstract     = {{<p>Introduction A second peak of inhibitors has been reported in patients with severe hemophilia A (HA) aged &gt;50 years in the United Kingdom. The reason for this suggested breakdown of tolerance in the aging population is unclear, as is the potential impact of regular exposure to the deficient factor by prophylaxis at higher age. No data on hemophilia B (HB) have ever been reported. Aim The ADVANCE Working Group investigated the incidence of late-onset inhibitors and the use of prophylaxis in patients with HA and HB aged ≥40 years. Methods A retrospective, observational, cohort, survey-based study of all patients aged ≥40 years with HA or HB treated at an ADVANCE hemophilia treatment center. Results Information on 3,095 people aged ≥40 years with HA or HB was collected. Of the 2,562 patients with severe HA, the majority (73% across all age groups) received prophylaxis. In patients with severe HA, the inhibitor incidence per 1,000 treatment years was 2.37 (age 40-49), 1.25 (age 50-59), and 1.45 (age 60 +). Overall, the inhibitor incidence was greatest in those with moderate HA (5.77 [age 40-49], 6.59 [age 50-59], and 4.69 [age 60 + ]) and the majority of inhibitor cases were preceded by a potential immune system challenge. No inhibitors in patients with HB were reported. Conclusion Our data do not identify a second peak of inhibitor development in older patients with hemophilia. Prophylaxis may be beneficial in older patients with severe, and possibly moderate HA, to retain a tolerant state at a higher age.</p>}},
  author       = {{Astermark, Jan and Ay, Cihan and Carvalho, Manuela and D'Oiron, Roseline and Moerloose, Philippe De and Dolan, Gerard and Fontana, Pierre and Hermans, Cedric and Holme, Pål Andre and Katsarou, Olga and Kenet, Gili and Klamroth, Robert and Mancuso, Maria Elisa and Marquardt, Natascha and Núñez, Ramiro and Pabinger, Ingrid and Tait, Robert and Valk, Paul Van Der}},
  issn         = {{0340-6245}},
  keywords     = {{aging; hemophilia A/B; inhibitors; prophylaxis}},
  language     = {{eng}},
  number       = {{6}},
  pages        = {{905--912}},
  publisher    = {{Georg Thieme Verlag}},
  series       = {{Thrombosis and Haemostasis}},
  title        = {{New Inhibitors in the Ageing Population : A Retrospective, Observational, Cohort Study of New Inhibitors in Older People with Hemophilia}},
  url          = {{http://dx.doi.org/10.1055/a-1642-4067}},
  doi          = {{10.1055/a-1642-4067}},
  volume       = {{122}},
  year         = {{2022}},
}