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Bone mineral density in haemophilia – a multicentre study evaluating the impact of different replacement regimens

Klintman, Jenny LU ; Åkesson, Kristina E LU ; Holme, Pål André and Fischer, Kathelijn (2022) In Haemophilia 28(2). p.239-246
Abstract
Aim
The aim of this study was to investigate if prophylactic treatment in severe haemophilia impact on bone mineral densisty (BMD) in adults with haemophilia A/B.
Methods
Subjects with haemophilia (n = 120) underwent bone-density measurement and clinical data was collected. BMD in subjects with severe haemophilia on high-dose prophylaxis (n = 41) was compared to BMD in subjects with mild haemophilia (n = 33) and to severe haemophilia treated with intermediate-dose prophylaxis (n = 32) or on-demand replacement therapy (n = 14).
Results
Subjects with severe haemophilia on high-dose prophylaxis showed BMD at total hip comparable to subjects with mild haemophilia (median BMD 955.8 and 977.4 mg/cm2 (P = .17), respectively).... (More)
Aim
The aim of this study was to investigate if prophylactic treatment in severe haemophilia impact on bone mineral densisty (BMD) in adults with haemophilia A/B.
Methods
Subjects with haemophilia (n = 120) underwent bone-density measurement and clinical data was collected. BMD in subjects with severe haemophilia on high-dose prophylaxis (n = 41) was compared to BMD in subjects with mild haemophilia (n = 33) and to severe haemophilia treated with intermediate-dose prophylaxis (n = 32) or on-demand replacement therapy (n = 14).
Results
Subjects with severe haemophilia on high-dose prophylaxis showed BMD at total hip comparable to subjects with mild haemophilia (median BMD 955.8 and 977.4 mg/cm2 (P = .17), respectively). No difference in BMD was found related to type of prophylactic regimen (median BMD 955.8 and 942.4 mg/cm2, in high-dose and intermediate dose groups, respectively; P = .70). Subjects with severe disease treated on-demand had significantly lower BMD compared to subjects on a high-dose prophylactic regimen (median BMD 771.8 and 955.8 mg/cm2 (P = .001), respectively). BMD decreased significantly with age, regardless of severity of haemophilia disease. In a multivariate analysis, adjusted for disease status and age, type of prophylactic regimen was not significantly associated with osteoporosis development.
Conclusion
We show that BMD differs in persons with severe haemophilia on propylaxis as compared to those treated on-demand, but that type of prophylactic regimen does not reflect on BMD. The difference between treatment groups was mainly explained by an age difference between groups. However, patients on prophylaxis displayed a high degree of normal BMD not far from mild haemophilia at comparative age.
(Less)
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author
; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Haemophilia
volume
28
issue
2
pages
239 - 246
publisher
Wiley-Blackwell
external identifiers
  • scopus:85122442043
  • pmid:34994489
ISSN
1351-8216
DOI
10.1111/hae.14487
language
English
LU publication?
yes
id
29fda843-9d73-44bf-bfc0-f1b5a0e822c0
date added to LUP
2022-01-10 15:03:08
date last changed
2024-05-30 09:21:59
@article{29fda843-9d73-44bf-bfc0-f1b5a0e822c0,
  abstract     = {{Aim<br/>The aim of this study was to investigate if prophylactic treatment in severe haemophilia impact on bone mineral densisty (BMD) in adults with haemophilia A/B.<br/>Methods<br/>Subjects with haemophilia (n = 120) underwent bone-density measurement and clinical data was collected. BMD in subjects with severe haemophilia on high-dose prophylaxis (n = 41) was compared to BMD in subjects with mild haemophilia (n = 33) and to severe haemophilia treated with intermediate-dose prophylaxis (n = 32) or on-demand replacement therapy (n = 14).<br/>Results<br/>Subjects with severe haemophilia on high-dose prophylaxis showed BMD at total hip comparable to subjects with mild haemophilia (median BMD 955.8 and 977.4 mg/cm2 (P = .17), respectively). No difference in BMD was found related to type of prophylactic regimen (median BMD 955.8 and 942.4 mg/cm2, in high-dose and intermediate dose groups, respectively; P = .70). Subjects with severe disease treated on-demand had significantly lower BMD compared to subjects on a high-dose prophylactic regimen (median BMD 771.8 and 955.8 mg/cm2 (P = .001), respectively). BMD decreased significantly with age, regardless of severity of haemophilia disease. In a multivariate analysis, adjusted for disease status and age, type of prophylactic regimen was not significantly associated with osteoporosis development.<br/>Conclusion<br/>We show that BMD differs in persons with severe haemophilia on propylaxis as compared to those treated on-demand, but that type of prophylactic regimen does not reflect on BMD. The difference between treatment groups was mainly explained by an age difference between groups. However, patients on prophylaxis displayed a high degree of normal BMD not far from mild haemophilia at comparative age.<br/>}},
  author       = {{Klintman, Jenny and Åkesson, Kristina E and Holme, Pål André and Fischer, Kathelijn}},
  issn         = {{1351-8216}},
  language     = {{eng}},
  month        = {{01}},
  number       = {{2}},
  pages        = {{239--246}},
  publisher    = {{Wiley-Blackwell}},
  series       = {{Haemophilia}},
  title        = {{Bone mineral density in haemophilia – a multicentre study evaluating the impact of different replacement regimens}},
  url          = {{http://dx.doi.org/10.1111/hae.14487}},
  doi          = {{10.1111/hae.14487}},
  volume       = {{28}},
  year         = {{2022}},
}