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Thromb Haemost 1987; 58(04): 1049-1052
DOI: 10.1055/s-0038-1646054
Original Article
Schattauer GmbH Stuttgart

Continuous High-Dose Factor VIII for the Induction of Immune Tolerance in Haemophilia A Patients with High Responder State: A Description of Eleven Patients Treated

E Scheibel
*   The University Departments of Paediatrics and Clinical Chemistry, Rigshospitalet, Copenhagen, Denmark
,
J lngerslev
**   The University Department of Ciinical Immunology, Aarhus Kommunehospital, Aarhus, Denmark
,
J Dalsgaard-Nielsen
***   The University Department of Haematology, Hvidovre Hospital, Hvidovre, Denmark
,
S Stenbjerg
**   The University Department of Ciinical Immunology, Aarhus Kommunehospital, Aarhus, Denmark
,
J B Knudsen
*   The University Departments of Paediatrics and Clinical Chemistry, Rigshospitalet, Copenhagen, Denmark
,
The Danish Study Group › Author Affiliations
Further Information

Publication History

Received 06 January 1986

Accepted after revision 07 September 1987

Publication Date:
29 June 2018 (online)

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Summary

Eleven severely affected haemophilia A patients (aged 6-42 y) with F VIII : C inhibitor (high responders) were treated with highdose F VIII in order to eliminate the inhibitors. The patients comprise Danish high responder patients treated during the period 1977-1985.

In all patients the inhibitors decreased significantly. In six, the inhibitor apparently disappeared (detection limit 0.4 Bethesda Units per ml) (BU/ml), in four patients a low level inhibitor of 0.4-1.4 BU/ml persisted. One patient is still on high-dose ;schedule. The duration of high-dose treatments ranged from less than one month up to 18 months. In all patients the tendency to spontaneous bleedings vanished when a measurable VIII : C level appeared in the post-infusion sample. The inhibitor suppression has allowed for extensive physical training and rehabilitation orthopaedic surgery. The patients are now able to conduct a normal haemophilic life on self-administered prophylactic doses of F VIII.

Keywords

Haemophilia A - High-dose F VIII - VIII :C inhibitor - High responder VIII:C inhibitor
 

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