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Successful treatment of high titer inhibitors in mild hemophilia A with avoidance of factor VIII and immunosuppressive therapy
Author(s) -
Robbins Daniel,
Kulkarni Roshni,
Gera Renuka,
ScottEmuakpor Ajovi B.,
Bosma Kathy,
Penner John A.
Publication year - 2001
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.1176
Subject(s) - desmopressin , medicine , titer , recombinant factor viia , complication , nasal administration , surgery , coagulopathy , anesthesia , immunology , antibody
We describe two patients with mild hemophilia A (MHA) who developed high titer inhibitor (HTI) following intensive recombinant factor VIII (rFVIII) concentrate replacement for surgery and trauma. Intranasal desmopressin was instituted shortly following immunosuppressive therapy (IST) and activated prothrombin complex concentrate (APCC) in one case, and following APCC alone in the second case. Avoidance of factor VIII (FVIII) coupled with intranasal desmopressin prophylaxis three times a week resulted in undetectable inhibitor 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. Am. J. Hematol. 68:184–188, 2001.© 2001 Wiley‐Liss, Inc.