Treatment of haemophilia A and B and von Willebrand's disease: summary and conclusions of a systematic review as part of a Swedish health-technology assessment

Malmo Centre for Thrombosis and Haemostasis, Skane University Hospital, Lund University, Malmo, Sweden.

Haemophilia. 2012;18((2):):158-65.
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In an ongoing health-technology assessment of haemophilia treatment in Sweden, performed by the governmental agency Dental and Pharmaceutical Benefits Agency (TLV; tandvards-och lakemedelsformansverket), the Swedish Council on Health Technology Assessment (SBU; statens beredning for medicinsk utvardering) was called upon to evaluate treatment of haemophilia A and B and von Willebrand's disease (VWD) with clotting factor concentrates. To evaluate the following questions: What are the short-term and long-term effects of different treatment strategies? What methods are available to treat haemophilia patients that have developed inhibitors against factor concentrates? Based on the questions addressed by the project, a systematic database search was conducted in PubMed, NHSEED, Cochrane Library, EMBASE and other relevant databases. The literature search covered all studies in the field published from 1985 up to the spring of 2010. In most instances, the scientific evidence is insufficient for the questions raised in the review. Concentrates of coagulation factors have good haemostatic effects on acute bleeding and surgical intervention in haemophilia A and B and VWD, but conclusions cannot be drawn about possible differences in the effects of different dosing strategies for acute bleeding and surgery. Prophylaxis initiated at a young age can prevent future joint damage in persons with haemophilia. The available treatment options for inhibitors have been insufficiently assessed. The economic consequences of various treatment regimens have been insufficiently analysed. Introduction of national and international registries is important. Copyright 2011 Blackwell Publishing Ltd. DO
Study details
Study Design : Systematic Review
Language : English
Additional Material : Discussion in: Miners AH (2013)Economic evaluations of prophylaxis with clotting factor for people with severe haemophilia: why do the results vary so much? Haemophilia 19(2) p174-80. PMID: 22989090
Credits : Bibliographic data from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine