title: Modification of biological parameters after treatment with recombinant factor VIIa in a patient with thrombocytopathy due to storage pool disease.
abstract: 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.
authors.affiliation: Department of Pediatrics, Division of Oncology-Hematology, Sophia Children's Hospital, University Hospital Rotterdam, Rotterdam, The Netherlands.
authors.forename: L
authors.initials: L
authors.lastname: Langendonck
authors.forename: I M
authors.initials: IM
authors.lastname: Appel
mesh_terms: D000293:Adolescent; D001791:Blood Platelet Disorders / Q000188:drug therapy*; D015942:Factor VIIa / Q000627:therapeutic use*; D015951:Factor Xa / Q000378:metabolism; D006801:Humans; D008297:Male; D010978:Platelet Factor 4 / Q000378:metabolism; D010981:Platelet Storage Pool Deficiency / Q000188:drug therapy*; D011994:Recombinant Proteins / Q000627:therapeutic use; D013917:Thrombin / Q000378:metabolism; D016896:Treatment Outcome
chemical_list: D011994:Recombinant Proteins; D010978:Platelet Factor 4; D015942:Factor VIIa; D013917:Thrombin; D015951:Factor Xa