[Inhibitor development after early high exposure and cerebral haemorrhage. Costs and factor demand for a successful immunotolerance induction therapy].
Severe haemophilia A was diagnosed postpartum in a newborn. The mother was known as a conductor (intron 22 inversion) and an uncle had a persistently high titer inhibitor after failed ITI. Due to a cephalhaematoma, a high-dose pdFVIII substitution was given within the first days after birth. At the age of six month a severe cerebral haemorrhage occurred, making a high-dose pdFVIII substitution and neurosurgical intervention necessary. Several days later a porth-a-cath-system was implanted. The development of a high titer inhibitor occured six days later, an ITI was started according to the Bonn Protocol. Initially rFVIIa was given in addition to the pdFVIII substitution. Seven days after the beginning of treatment the inhibitor was no longer detectable. At monthly intervals the FVIII dosage was reduced until the dosage complied with a prophylaxis in severe haemophilia A. The duration of the ITI was nine months. A total of 30 mg rFVIIa and 276000 IU pdFVIII were used; costs in total: 280173.60 Euro.
Kinderklinik; Klinikum Bremen Mitte, Sankt-Jürgen-Straße 1, 28205 Bremen. kirstinhaubold@yahoo.com
K
K
Haubold
C
C
Moorthi
A
A
Bade
C
C
Niekrens
G
G
Auerswald
D017281:Cost of Illness; D004305:Dose-Response Relationship, Drug; D005169:Factor VIII / Q000191:economics / Q000627:therapeutic use*; D015942:Factor VIIa / Q000191:economics / Q000627:therapeutic use; D005260:Female; D006406:Hematoma / Q000188:drug therapy / Q000209:etiology; D006467:Hemophilia A / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D007438:Introns / Q000235:genetics; D011994:Recombinant Proteins / Q000191:economics / Q000627:therapeutic use
D011994:Recombinant Proteins; D005169:Factor VIII; C103587:recombinant FVIIa; D015942:Factor VIIa