The Problems of Inhibitor in Children Hemophilic Patients Observed at the Pediatric Hematology and Oncology Clinic, University Hospital Prague-Motol

Overview

Among 230 pediatric hemophiliacs registered by 31st March 2007 in the HemIS register (Hemophilia – Information System), there are 26 patients (11.3%) with a history of inhibitor, and only 6 of them (2.3%) have the evidence of an active inhibitor requiring treatment. Between 1990 and 2006 in the Second Department of Pediatrics and later in the Department of Pediatric Hematology and Oncology of the University Hospital Motol, 19 patients with an inhibitor were followed up, thirteen of them were treated with the objective of inhibitor elimination. Immunosuppressive and immune tolerance therapy was used. In 10 of 13 treated patients the therapy led to a permanent elimination of inhibitor, in 3 patients it did not succeed due to an early termination of therapy. One of these patients died at home due to an unrecognized retroperitoneal bleeding. A treatment algorithm based on our experience is proposed in the discussion.

Key words:
register HemIS, pediatric hemophiliacs, inhibitor, immunosuppressive treatment, immune tolerance treatment