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Alternative treatment options for pediatric hemophilia B patients with high-responding inhibitors: A thrombin

Assaf Arie Barg1,2, Sarina Levy-Mendelovich1,2, Einat Avishai2

  • 1Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Pediatric Blood & Cancer
|September 20, 2018
PubMed

Little is known about the challenging treatment of pediatric patients with hemophilia B and inhibitors due to disease rarity. We describe three patients diagnosed in childhood and followed up to 9 years. All three had allergic reactions to Factor IX, but two were later safely treated for bleeding episodes with activated prothrombin complex concentrates (APCC = FEIBA). The third was given only recombinant activated Factor VIIa. Based on ex vivo thrombin generation analysis, a new alternative treatment of combined bypassing agents was administered for bleeding episodes and several minor surgical procedures with no treatment-associated adverse events or thrombosis.

Keywords:
FEIBAhemophilia BinhibitorrFVIIathrombin generation

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