Successful treatment of high titer inhibitors in mild hemophilia a with avoidance of factor VIII and immunosuppressive therapy

Citation
D. Robbins et al., Successful treatment of high titer inhibitors in mild hemophilia a with avoidance of factor VIII and immunosuppressive therapy, AM J HEMAT, 68(3), 2001, pp. 184-188
Citations number
40
Categorie Soggetti
Hematology,"Cardiovascular & Hematology Research
Journal title
AMERICAN JOURNAL OF HEMATOLOGY
ISSN journal
03618609 → ACNP
Volume
68
Issue
3
Year of publication
2001
Pages
184 - 188
Database
ISI
SICI code
0361-8609(200111)68:3<184:STOHTI>2.0.ZU;2-1
Abstract
We describe two patients with mild hemophilia A (MHA) who developed high ti ter inhibitor (HTI) following intensive recombinant factor VIII (rFVIII) co ncentrate replacement for surgery and trauma. Intranasal desmopressin was i nstituted shortly following Immunosuppressive therapy (IST) and activated p rothrombin complex concentrate (APCC) in one case, and following APCC alone in the second case. Avoidance of factor VIII (FVIII) coupled with intranas al desmopressin prophylaxis three times a week resulted In undetectable inh ibitor levels. Both patients have had no further bleeding episodes and have been maintained on desmopressin prophylaxis prior to activity for the past 2 to 3 years. Recombinant factor VIIa (rFVIIa) was used successfully prior to a second surgery In one patient without complication. (C) 2001 Wiley-Li ss, Inc.