Immune tolerance induction in a severe hemophilia A patient with inhibitor.
- Author:
Lei ZHANG
1
;
Feng XUE
;
Xiao-Fan LIU
;
Ze-Ping ZHOU
;
Yong-Ze LIU
;
Meng-Su TIAN
;
Lin SHEN
;
Xian-Hui XU
;
Hong-Li ZHANG
;
Ren-Chi YANG
Author Information
- Publication Type:Case Reports
- MeSH: Autoantibodies; immunology; Factor VIII; genetics; Hemophilia A; genetics; Humans; Immune Tolerance; drug effects; Immunosuppression
- From: Chinese Journal of Hematology 2010;31(9):577-580
- CountryChina
- Language:Chinese
-
Abstract:
OBJECTIVETo explore the immune tolerance induction (ITI) in a severe hemophilia A patient with inhibitor, and to improve the therapeutic efficacy for patient.
METHODSThe FVIII:C was assayed by one-stage method and FVIII antibody by Bethesda method. Mutation screening of FVIII gene intron 22 inversion was performed using LD-PCR.
RESULTSFVIII gene intron 22 inversion was detected in this patient. Clinical tolerance to FVIII was successfully induced after administration of the ITI regimen combined with immunosuppression. A fall of inhibitor titer from 8 BU to 0 BU after treatment for 3 months, and in vivo FVIII recovery (> 66%) was normalized. The patient had no bleeding episode in the following 6 months.
CONCLUSIONThis is the first case report on successful immune tolerance induction therapy in Chinese hemophilia A patient. ITI is the most effective therapy for hemophilia A with inhibitor.