Patients with thrombocytopathy due to storage pool disease mostly suffer from mild bleeding diathesis. However surgical interventions can lead to excess bleeding. We describe how treatment with recombinant factor VIIa (Novoseven®) during a surgical procedure in a boy with SPD leads to an immediate rise in PF-4, thereby activating factor Xa on the platelet surface, leading to active thrombin generation.

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doi.org/10.1002/pbc.20199, hdl.handle.net/1765/73243
Pediatric Blood & Cancer
Department of Pediatrics

Langendonck, L., & Appel, I. (2005). Modification of biological parameters after treatment with recombinant factor VIIa in a patient with thrombocytopathy due to storage pool disease. In Pediatric Blood & Cancer (Vol. 44, pp. 676–678). doi:10.1002/pbc.20199