haemophilia A;
factor VIII;
factor VIII inhibitors;
catalytic antibodies;
D O I:
10.1097/00001721-200306001-00008
中图分类号:
R5 [内科学];
学科分类号:
1002 ;
100201 ;
摘要:
The development of factor VIII (FVIII) inhibitors in haemophiliac patients following therapeutic administration of exogenous FVIII is one of the major factors complicating the treatment of this disease. Most FVII inhibitors described to date appear to be directed towards epitopes; involved in the procoagulant activity of FVIII. However, recent data suggest that some FVIII inhibitors may behave as catalytic antibodies, able to cleave FVIII by hydrolysis. This appears to be the first example of catalytic antibodies having a direct role in the etiology of a disease. Further characterization of these catalytic anti-FVIII antibodies may lead to the identification of novel therapeutic approaches for the future management of FVIII inhibitor patients. (C) 2003 Lippincott Williams Wilkins.
机构:
INSERM, U872, Paris, France
Univ Paris 06, Ctr Rech Cordeliers Cordeliers, UMR S 872, F-75006 Paris, France
Univ Paris 05, UMR S 872, Paris, FranceCHU Caen, Dept Hematol, F-14033 Caen, France
Kaveri, S. V.
Borel-Derlon, A.
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机构:
CHU Caen, Dept Hematol, F-14033 Caen, France
Univ Caen Basse Normandie, Sch Med, Caen, France
INSERM, U919, Serine Proteases & Pathophysiol Neurovasc Unit, Caen, FranceCHU Caen, Dept Hematol, F-14033 Caen, France
Borel-Derlon, A.
Repesse, Y.
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机构:
CHU Caen, Dept Hematol, F-14033 Caen, France
Univ Caen Basse Normandie, Sch Med, Caen, France
INSERM, U919, Serine Proteases & Pathophysiol Neurovasc Unit, Caen, FranceCHU Caen, Dept Hematol, F-14033 Caen, France
机构:
QMUL, Royal London Hosp, Haemophilia Ctr, Barts & London Sch Med & Dent, London, EnglandQMUL, Royal London Hosp, Haemophilia Ctr, Barts & London Sch Med & Dent, London, England
Batty, Paul
Moore, Gary W.
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机构:
Viapath Guys & St Thomas NHS Fdn Trust, Haemostasis & Thrombosis, London, EnglandQMUL, Royal London Hosp, Haemophilia Ctr, Barts & London Sch Med & Dent, London, England
Moore, Gary W.
Platton, Sean
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机构:
Royal London Hosp, Haemophilia Ctr, Barts Hlth NHS Trust, London E1 1BB, EnglandQMUL, Royal London Hosp, Haemophilia Ctr, Barts & London Sch Med & Dent, London, England
Platton, Sean
Maloney, James C.
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机构:
Viapath Guys & St Thomas NHS Fdn Trust, Haemostasis & Thrombosis, London, EnglandQMUL, Royal London Hosp, Haemophilia Ctr, Barts & London Sch Med & Dent, London, England
Maloney, James C.
Palmer, Ben
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机构:
United Kingdom Natl Haemophilia Database, Manchester, Lancs, EnglandQMUL, Royal London Hosp, Haemophilia Ctr, Barts & London Sch Med & Dent, London, England
Palmer, Ben
Bowles, Louise
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机构:
Royal London Hosp, Haemophilia Ctr, Barts Hlth NHS Trust, London E1 1BB, EnglandQMUL, Royal London Hosp, Haemophilia Ctr, Barts & London Sch Med & Dent, London, England
Bowles, Louise
Pasi, K. John
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机构:
QMUL, Royal London Hosp, Haemophilia Ctr, Barts & London Sch Med & Dent, London, EnglandQMUL, Royal London Hosp, Haemophilia Ctr, Barts & London Sch Med & Dent, London, England
Pasi, K. John
Rangarajan, Savita
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机构:
Guys & St Thomas NHS Fdn Trust, Ctr Haemostasis & Thrombosis, London, EnglandQMUL, Royal London Hosp, Haemophilia Ctr, Barts & London Sch Med & Dent, London, England
Rangarajan, Savita
Hart, Daniel P.
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机构:
QMUL, Royal London Hosp, Haemophilia Ctr, Barts & London Sch Med & Dent, London, EnglandQMUL, Royal London Hosp, Haemophilia Ctr, Barts & London Sch Med & Dent, London, England