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KMID : 0366220160510010037
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Korean Journal of Hematology
2016 Volume.51 No. 1 p.37 ~ p.43
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Long-term course of anti-factor VIII antibody in patients with hemophilia A at a single center
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Yoo Ki-Young
Joo Sang-Chun
Choi Yong-Mook
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Abstract
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BackgroundI: mmune tolerance induction (ITI) can reduce inhibitors against factor VIII concentrates by 70?80%. In this study, we elucidated the characteristics of inhibitors and attempted to determine the proper indications and timing for ITI.
Methods: Subjects included hemophilia A patients registered at the Korea Hemophilia Foundation from 1991 through 2014. Inhibitors were classified as persistent and transient. Patients were classified into groups according to peak inhibitor titer: low (＜2 BU/mL), moderate (2 to ＜5 BU/mL), high (5 to ＜10 BU/mL), and very high titer (≥10 BU/mL).
Results: Overall, 350 (21.4%) of 1,634 hemophilia A patients developed inhibitors at least once. Of these, 100 (6.1%) and 250 (15.3%) patients developed persistent and transient inhibitors, respectively. For transient inhibitors, the median peak titer was 1.0 BU/mL, persistent for median of 11.0 months (10.0, 8.0, 13.0, and 19.0 months in the low, moderate, high, and very high titer transient inhibitor groups, respectively). Overall, 95.8% (215), 72.2% (17), 52.4% (21), and 21.7% (97) of patients in the low, moderate, high, and very high titer groups became inhibitor-negative spontaneously, without ITI.
Conclusion: Given the spontaneous disappearance of inhibitors and high cost of ITI, it is worthwhile to postpone ITI for 11 months unless the peak inhibitor titer is greater than 10 BU/mL.
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KEYWORD
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Hemophilia A, Inhibitor, Longitudinal study
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