Haemophilia;
secondary prophylaxis;
adult;
bleeding rate;
factor VIII consumption;
D O I:
10.5482/HAMO-12-07-0013
中图分类号:
R5 [内科学];
学科分类号:
1002 ;
100201 ;
摘要:
Primary prophylaxis has been proven as the gold standard for preserving joint function in severe haemophilia. Secondary prophylaxis in children has also been shown to have a more beneficial effect on joint protection as on-demand treatment. The outcome of delayed secondary prophylaxis started in adulthood is rarely studied. The case of a man (age: 47 years) with severe haemophilia A demonstrates the successful switch from on-demand therapy to prophylaxis with subsequent reduction of annual bleeding rate and number of days of work lost. The small increase of factor VIII consumption (16%) and the decrease of bleeding rate are compared with data from two recently published prospective studies. Conclusion: An optimal dosing schedule needs to be discussed and larger and longer prospective studies targeting the benefits of secondary prophylaxis with factor VIII started in adulthood are necessary.
机构:
Versiti Blood Res Inst, Milwaukee, WI USA
Med Coll Wisconsin, Dept Med, Div Hematol & Oncol, Milwaukee, WI USA
Med Coll Wisconsin, Dept Pediat, Div Hematol & Oncol, Milwaukee, WI USA
8733 W Watertown Plank Rd, Milwaukee, WI 53226 USAVersiti Blood Res Inst, Milwaukee, WI USA
Malec, Lynn
Matino, Davide
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机构:
McMaster Univ, Dept Med, Div Hematol & Thromboembolism, Hamilton, ON, CanadaVersiti Blood Res Inst, Milwaukee, WI USA
机构:
Department of Paediatrics, Haematology and Oncology, Warsaw Medical University, 00-576 WarsawDepartment of Paediatrics, Haematology and Oncology, Warsaw Medical University, 00-576 Warsaw
Laguna P.
Klukowska A.
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h-index: 0
机构:
Department of Paediatrics, Haematology and Oncology, Warsaw Medical University, 00-576 WarsawDepartment of Paediatrics, Haematology and Oncology, Warsaw Medical University, 00-576 Warsaw