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German recommendations for immune tolerance therapy in type A haemophiliacs with antibodies
Author(s) -
Brackmann,
Lenk,
Scharrer,
Auerswald,
Kreuz
Publication year - 1999
Publication title -
haemophilia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.213
H-Index - 92
eISSN - 1365-2516
pISSN - 1351-8216
DOI - 10.1046/j.1365-2516.1999.00311.x
Subject(s) - medicine , haemophilia , haemophilia a , antibody , immune tolerance , immune system , coagulation , immunology , surgery
Haemophilia A is the most common X‐chromosomal‐linked congenital bleeding disorder and is caused by decreased activity of blood coagulation factor VIII. Affected individuals develop a variable phenotype of haemorrhages, mainly into joints and muscles depending on the amount of the residual factor VIII. The exogenous factor VIII‐substitution by plasma‐derived or recombinant products are the only treatments either on demand or prophylactically. The most important complication of treatment is the development of inhibitors that affect about 20%–50% of the severe cases. These antibodies neutralize the therapeutic effect of factor VIII‐concentrates, leading to recurrent bleeding episodes, progredient joint damages and sometimes life‐threatening situations. The only chance for a complete and permanent eradication of the inhibitors in these patients is the induction of Immune‐Tolerance (ITT) to substituted factor VIII by the application of high‐doses of factor VIII. The treatment demands a strict compliance of the patient and a much higher effort of the physician, to non‐compared inhibitor patients. Requirements for a consistent realization of the ITT to increase the successful outcome was carried out by German Haemophilia Center Directors.