Successful treatment of high titer inhibitors in mild hemophilia A with avoidance of factor VIII and immunosuppressive therapy.
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.
Robbins
D
D
Michigan State University, Department of Pediatrics/Human Development, B220 Clinical Center, 138 Service Road, East Lansing, Michigan 48824-1313, USA.
Kulkarni
R
R
Gera
R
R
Scott-Emuakpor
A B
AB
Bosma
K
K
Penner
J A
JA
D000293:Adolescent; D001323:Autoantibodies / Q000097:blood / Q000187:drug effects*; D000075202:Contraindications; D003894:Deamino Arginine Vasopressin / Q000008:administration & dosage; D005169:Factor VIII / Q000187:drug effects / Q000276:immunology*; D006467:Hemophilia A / Q000188:drug therapy* / Q000276:immunology*; D006490:Hemostatics / Q000008:administration & dosage; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008297:Male; D016896:Treatment Outcome
D001323:Autoantibodies; D006490:Hemostatics; D007166:Immunosuppressive Agents; D005169:Factor VIII; D003894:Deamino Arginine Vasopressin