Anaphylactoid reactions and nephrotic syndrome - A considerable risk during factor IX treatment in patients with haemophilia B and inhibitors : A report on the outcome in two brothers
Tengborn, L. LU ; Hansson, S. LU ; Fasth, A. ; Lübeck, P. O. ; Berg, A. LU and Ljung, R. LU
(1998)
In Haemophilia
4(6).
p.854-859
- Abstract
Anaphylaxis/anaphylactoid reactions have recently been reported after few treatments with factor IX concentrates in patients with haemophilia B at the same time as inhibitors to factor IX were demonstrated. In some of these cases nephrotic syndrome has appeared during immune tolerance induction (ITI) with high doses of factor IX concentrates. Gene deletions seem to be associated with a high risk of developing antibodies to factor IX. This report presents two brothers with deletion of 1 bp in exon f of the factor IX gene. Both showed anaphylactoid reactions and they were desensitized using slow i.v. injections of factor IX. At the time of anaphylaxis, inhibitors of factor IX in a low titre could be demonstrated. The elder brother... (More)
Anaphylaxis/anaphylactoid reactions have recently been reported after few treatments with factor IX concentrates in patients with haemophilia B at the same time as inhibitors to factor IX were demonstrated. In some of these cases nephrotic syndrome has appeared during immune tolerance induction (ITI) with high doses of factor IX concentrates. Gene deletions seem to be associated with a high risk of developing antibodies to factor IX. This report presents two brothers with deletion of 1 bp in exon f of the factor IX gene. Both showed anaphylactoid reactions and they were desensitized using slow i.v. injections of factor IX. At the time of anaphylaxis, inhibitors of factor IX in a low titre could be demonstrated. The elder brother responded well after a short time on ITI and has no spontaneous bleedings on regular prophylaxis although in a somewhat higher dose than expected. On the other hand, in spite of comparable regimens, the younger brother has so far been resistant to ITI. Moreover, during treatment with extremely high doses of factor IX concentrate he developed nephrotic syndrome which only slowly subsided after treatment with corticosteroids and withdrawal of factor IX.
(Less)
- author
- Tengborn, L.
LU
; Hansson, S.
LU
; Fasth, A.
; Lübeck, P. O.
; Berg, A.
LU
and Ljung, R.
LU
- organization
- publishing date
- 1998
- type
- Contribution to journal
- publication status
- published
- keywords
- Anaphylactoid reactions, Haemophilia B, Inhibitors of factor IX, Nephrotic syndrome
- in
- Haemophilia
- volume
- 4
- issue
- 6
- pages
- 854 - 859
- publisher
- Wiley-Blackwell
- external identifiers
-
- scopus:0031743696
- pmid:10028310
- ISSN
- 1351-8216
- DOI
- 10.1046/j.1365-2516.1998.00190.x
- language
- English
- LU publication?
- yes
- id
- 1e9e7fa6-4990-4ef4-af96-b18ff9968d5d
- date added to LUP
- 2016-11-08 15:18:56
- date last changed
- 2024-02-03 03:42:49
@article{1e9e7fa6-4990-4ef4-af96-b18ff9968d5d,
abstract = {{<p>Anaphylaxis/anaphylactoid reactions have recently been reported after few treatments with factor IX concentrates in patients with haemophilia B at the same time as inhibitors to factor IX were demonstrated. In some of these cases nephrotic syndrome has appeared during immune tolerance induction (ITI) with high doses of factor IX concentrates. Gene deletions seem to be associated with a high risk of developing antibodies to factor IX. This report presents two brothers with deletion of 1 bp in exon f of the factor IX gene. Both showed anaphylactoid reactions and they were desensitized using slow i.v. injections of factor IX. At the time of anaphylaxis, inhibitors of factor IX in a low titre could be demonstrated. The elder brother responded well after a short time on ITI and has no spontaneous bleedings on regular prophylaxis although in a somewhat higher dose than expected. On the other hand, in spite of comparable regimens, the younger brother has so far been resistant to ITI. Moreover, during treatment with extremely high doses of factor IX concentrate he developed nephrotic syndrome which only slowly subsided after treatment with corticosteroids and withdrawal of factor IX.</p>}},
author = {{Tengborn, L. and Hansson, S. and Fasth, A. and Lübeck, P. O. and Berg, A. and Ljung, R.}},
issn = {{1351-8216}},
keywords = {{Anaphylactoid reactions; Haemophilia B; Inhibitors of factor IX; Nephrotic syndrome}},
language = {{eng}},
number = {{6}},
pages = {{854--859}},
publisher = {{Wiley-Blackwell}},
series = {{Haemophilia}},
title = {{Anaphylactoid reactions and nephrotic syndrome - A considerable risk during factor IX treatment in patients with haemophilia B and inhibitors : A report on the outcome in two brothers}},
url = {{http://dx.doi.org/10.1046/j.1365-2516.1998.00190.x}},
doi = {{10.1046/j.1365-2516.1998.00190.x}},
volume = {{4}},
year = {{1998}},
}