We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Nephrotic syndrome in two haemophilia B children with inhibitor under low‐dose immune tolerance induction combined with rituximab‐based immunosuppressant protocol.
- Authors
Li, Zekun; Liu, Guoqing; Yao, Wanru; Chen, Zhenping; Li, Gang; Cheng, Xiaoling; Zhen, Yingzi; Ai, Di; Huang, Kun; Poon, Man‐Chiu; Wu, Runhui
- Abstract
Allergic reactions to FIX have been associated with nephrotic syndrome and poor ITI outcome.3 Both our cases did, with case 2 having allergic reactions simultaneously with inhibitor development. This is consistent with previous observations that NS development was closely related to a significant decline in inhibitor titre.2 Interestingly, the NS in case 2 was preceded by a second allergic reactions to the ITI regimen and in case 1 by a fluctuation of inhibitor titres during ITI-rituximab. Patients with haemophilia B (HB) may develop factor IX (FIX) inhibitors with a cumulative incidence of 10.2% following FIX replacement therapy by 500 exposure days.1 In 50-60% cases, occurrence of severe allergic reactions may precede or occur simultaneously with the inhibitor development.2 The propensity for FIX to induce allergic reactions (compared to FVIII) may be related to the extravascular distribution of the smaller FIX molecules.3 Immune tolerance induction (ITI) therapy to eradicate FIX inhibitors has a poor success rate of only 13-31%.2,4 There is also a risk of developing nephrotic syndrome (NS) in 17-33% cases. Nephrotic syndrome in two haemophilia B children with inhibitor under low-dose immune tolerance induction combined with rituximab-based immunosuppressant protocol.
- Subjects
IMMUNOLOGICAL tolerance; NEPHROTIC syndrome; HEMOPHILIA; VON Willebrand disease; MALIGNANT hyperthermia
- Publication
Haemophilia, 2022, Vol 28, Issue 2, pe42
- ISSN
1351-8216
- Publication type
Article
- DOI
10.1111/hae.14471