Inhibitors in the Swedish population with severe haemophilia A and B : A 20-year survey
Knobe, Karin LU ; Sjörin, Elsy LU ; Tengborn, Lilian LU ; Petrini, P and Ljung, Rolf LU
(2002)
In Acta Pædiatrica
91(8).
p.910-914
- Abstract
Aim: To survey the entire population (n = 116) afflicted with severe haemophilia A or B born in Sweden over a 20-y period (1980-1999), and to examine the epidemiological, genetic and clinical aspects of development of inhibitors to factors VIII and IX (FVIII/FIX). Methods: One hundred of the subjects had haemophilia A and 16 had haemophilia B. All of these subjects had received prophylactic treatment and had a check-up of inhibitor status at least twice a year. Sixty-one were born between 1980 and 1989 and 55 between 1990 and 1999. Results: Nineteen percent (19/100) of those with haemophilia A and 37% (6/16) with haemophilia B developed inhibitors at 12-18 mo of age, after exposure to FVIII/FIX concentrates for an average of 14d in the... (More)
Aim: To survey the entire population (n = 116) afflicted with severe haemophilia A or B born in Sweden over a 20-y period (1980-1999), and to examine the epidemiological, genetic and clinical aspects of development of inhibitors to factors VIII and IX (FVIII/FIX). Methods: One hundred of the subjects had haemophilia A and 16 had haemophilia B. All of these subjects had received prophylactic treatment and had a check-up of inhibitor status at least twice a year. Sixty-one were born between 1980 and 1989 and 55 between 1990 and 1999. Results: Nineteen percent (19/100) of those with haemophilia A and 37% (6/16) with haemophilia B developed inhibitors at 12-18 mo of age, after exposure to FVIII/FIX concentrates for an average of 14d in the case of haemophilia A and 16 d in haemophilia B. All patients with inhibitors carried mutations that impaired protein synthesis. The high incidence of FIX inhibitors may have been due to the large number of complete deletions (13%) in the Swedish haemophilia B population. Patients with haemophilia A showed no significant increase (p = 0.65) in incidence of inhibitors (n = 10/48, total incidence 21%) in the 1990s, when they were treated mainly with recombinant products, as compared to the 1980s (n=9/52, 17%) when they received intermediate/high-purity plasma-derived concentrates. Conclusion: Our population-based study verifies that genotype has a general impact on the incidence of FVIII/FIX inhibitors, and that recombinant FIII/FIX concentrates are not a predisposing factor for inhibitor development.
(Less)
- author
- Knobe, Karin
LU
; Sjörin, Elsy
LU
; Tengborn, Lilian
LU
; Petrini, P
and Ljung, Rolf
LU
- publishing date
- 2002
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- Factor IX, Factor VIII, Haemophilia A, Haemophilia B, Inhibitor
- in
- Acta Pædiatrica
- volume
- 91
- issue
- 8
- pages
- 5 pages
- publisher
- Wiley-Blackwell
- external identifiers
-
- wos:000177409600011
- pmid:12222714
- scopus:0036044248
- pmid:12222714
- ISSN
- 1651-2227
- DOI
- 10.1080/080352502760148621
- language
- English
- LU publication?
- no
- additional info
- The information about affiliations in this record was updated in December 2015. The record was previously connected to the following departments: Emergency medicine/Medicine/Surgery (013240200), Paediatric Hematologic Research Group (013243020)
- id
- a938daa8-c0b1-4156-a04e-cb7104c03f73 (old id 114886)
- date added to LUP
- 2016-04-01 16:53:30
- date last changed
- 2022-01-28 22:55:40
@article{a938daa8-c0b1-4156-a04e-cb7104c03f73,
abstract = {{<p>Aim: To survey the entire population (n = 116) afflicted with severe haemophilia A or B born in Sweden over a 20-y period (1980-1999), and to examine the epidemiological, genetic and clinical aspects of development of inhibitors to factors VIII and IX (FVIII/FIX). Methods: One hundred of the subjects had haemophilia A and 16 had haemophilia B. All of these subjects had received prophylactic treatment and had a check-up of inhibitor status at least twice a year. Sixty-one were born between 1980 and 1989 and 55 between 1990 and 1999. Results: Nineteen percent (19/100) of those with haemophilia A and 37% (6/16) with haemophilia B developed inhibitors at 12-18 mo of age, after exposure to FVIII/FIX concentrates for an average of 14d in the case of haemophilia A and 16 d in haemophilia B. All patients with inhibitors carried mutations that impaired protein synthesis. The high incidence of FIX inhibitors may have been due to the large number of complete deletions (13%) in the Swedish haemophilia B population. Patients with haemophilia A showed no significant increase (p = 0.65) in incidence of inhibitors (n = 10/48, total incidence 21%) in the 1990s, when they were treated mainly with recombinant products, as compared to the 1980s (n=9/52, 17%) when they received intermediate/high-purity plasma-derived concentrates. Conclusion: Our population-based study verifies that genotype has a general impact on the incidence of FVIII/FIX inhibitors, and that recombinant FIII/FIX concentrates are not a predisposing factor for inhibitor development.</p>}},
author = {{Knobe, Karin and Sjörin, Elsy and Tengborn, Lilian and Petrini, P and Ljung, Rolf}},
issn = {{1651-2227}},
keywords = {{Factor IX; Factor VIII; Haemophilia A; Haemophilia B; Inhibitor}},
language = {{eng}},
number = {{8}},
pages = {{910--914}},
publisher = {{Wiley-Blackwell}},
series = {{Acta Pædiatrica}},
title = {{Inhibitors in the Swedish population with severe haemophilia A and B : A 20-year survey}},
url = {{http://dx.doi.org/10.1080/080352502760148621}},
doi = {{10.1080/080352502760148621}},
volume = {{91}},
year = {{2002}},
}