German recommendations for immune tolerance therapy in type A haemophiliacs with antibodies.
Haemophilia
; 5(3): 203-6, 1999 May.
Article
en En
| MEDLINE
| ID: mdl-10444288
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.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Factor VIII
/
Hemofilia A
/
Tolerancia Inmunológica
Tipo de estudio:
Guideline
Límite:
Humans
País/Región como asunto:
Europa
Idioma:
En
Revista:
Haemophilia
Asunto de la revista:
HEMATOLOGIA
Año:
1999
Tipo del documento:
Article
País de afiliación:
Alemania
Pais de publicación:
Reino Unido