Secondary prophylaxis in an adult patient with severe haemophilia A

Author:

Schubert C.,Schlenkrich S.

Abstract

SummaryPrimary 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 sub-sequent 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.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.

Funder

Andrea Trawinski and Bayer Vital for medical writing assistance

Publisher

Georg Thieme Verlag KG

Subject

Hematology

Reference14 articles.

1. Guidelines for the Management of Haemophilia. World Federation of haemophilia 2005. www.wfh.org/2/docs/publications/diagnosis_and_Treatment/Guidelines_Mng_Haemophilia.pdf

2. Querschnitts Leitlinien (BÄK) zur Therapie mit Blutkomponenten und Plasmaderivaten. 4. Auf. 2008. www.bundesaerztekammer.de/downloads/Blutkomponente_Querschnittsleitlinie

3. National Haemophilia Foundation. MASAC Recommendations concerning prophylaxis (regular administration of clotting factor concentrate to prevent bleeding). Document 179. www.haemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=57contentid=1007 (last access on February 2012)

4. Prophylaxis in Children with Hemophilia: Evidence-Based Achievements, Old and New Challenges

5. Assessing the effectiveness and cost-effectiveness of prophylaxis against bleeding in patients with severe haemophilia and severe von Willebrand's disease

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