Blood Res.  2019 Sep;54(3):204-209. 10.5045/br.2019.54.3.204.

The prevalence and risk factors of inhibitor development of FVIII in previously treated patients with hemophilia A

Affiliations
  • 1Department of Pediatrics, Eulji University School of Medicine, Daejeon, Korea. YCW1@eulji.ac.kr

Abstract

BACKGROUND
Risk factors for the development of inhibitors in previously untreated patients (PUPs) have been reported; this is not the case in previously treated patients (PTPs) owing to fewer studies. Risk factors may differ for the development of PTP versus PUP inhibitors. We aimed to identify risk factors for PTP inhibitor development.
METHODS
Participants were patients at a hemophilia treatment center in Korea with current or past history of factor VIII or factor IX alloantibodies. Observed inhibitors were classified as PUP or PTP inhibitors based on the cumulative number of exposure days. We compared the type and severity of hemophilia, mutation type, and family history of inhibitor between PUPs and PTPs. Events within 3 months before the first inhibitor detection, such as change of the factor concentrate used, short-term high exposure or continuous infusion of factor concentrate, history of surgery, infection, diagnosis of cancer, use of immunosuppressive or immunomodulator agents, and vaccination were compared between PUPs and PTPs.
RESULTS
We observed 5 PUP inhibitors and 5 PTP inhibitors in 115 patients with hemophilia A. Events that might be related to the development of inhibitors within 3 months prior to the first inhibitor detection were observed in all 5 PTPs. On the contrary, no such events were observed in any PUPs. The observed events included a change in the factor concentrate used, subsequent chemotherapy, and short-term high exposure to factor concentrates for controlling hemorrhage and surgeries.
CONCLUSION
Our results suggest a greater role of nongenetic factors in PTP inhibitor development.

Keyword

Hemophilia A; Previously treated patient; Inhibitor; Risk factor

MeSH Terms

Diagnosis
Drug Therapy
Factor IX
Factor VIII
Hemophilia A*
Hemorrhage
Humans
Isoantibodies
Korea
Prevalence*
Risk Factors*
Vaccination
Factor IX
Factor VIII
Isoantibodies
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