title: [Postpartum hemorrhage successfully treated with recombinant factor VIIa in Glanzmann thromboasthenia].
abstract: Glanzmann thrombasthenia is an inherited hemorrhagic disorder characterized by severe reduction or absence of platelet aggregation in response to multiple physiologic agonists due to qualitative or quantitative abnormalities of platelet glycoprotein (GP) IIb/IIIa. Treatment of bleeding episodes may require platelet transfusion. However, repeated platelet transfusions may result in GPIIb/IIIa and/or HLA immunization, and development of platelet refractoriness. Recently, a number of reports have suggested that recombinant factor VIIa product (rFVIIa) may be a therapeutic alternative in these situations. We have used rFVIIa to treat a 34-year-old primigravida woman for postpartum bleeding. She had been diagnosed with Glanzmann thrombasthenia at 32 years of age. At 37 weeks gestation, she underwent elective caesarean section uneventfully with platelet transfusion. Nine days later, she developed vaginal bleeding and received twenty units of platelet concentrates every day, but bleeding persisted. Thereafter, 4.8 mg intravenous rFVIIa was administered three times, which immediately arrested the bleeding. These results demonstrate that rFVIIa is effective for severe bleeding in Glanzmann thrombasthenia patients, especially in those with antiplatelet antibodies and/or platelet transfusion refractoriness.
authors.affiliation: Department of Hematology and Oncology, Division of Clinical and Experimental Oncology, Research Institute for Radiation Biology and Medicine, Hiroshima University.
authors.forename: Sayaka
authors.initials: S
authors.lastname: Sugihara
authors.forename: Shinya
authors.initials: S
authors.lastname: Katsutani
authors.forename: Hideo
authors.initials: H
authors.lastname: Hyodo
authors.forename: Maki
authors.initials: M
authors.lastname: Hyodo
authors.forename: Yoshiki
authors.initials: Y
authors.lastname: Kudo
authors.forename: Teruhisa
authors.initials: T
authors.lastname: Fujii
authors.forename: Akiro
authors.initials: A
authors.lastname: Kimura
mesh_terms: D000328:Adult; D002585:Cesarean Section; D015942:Factor VIIa / Q000008:administration & dosage*; D005260:Female; D006801:Humans; D006473:Postpartum Hemorrhage / Q000188:drug therapy* / Q000209:etiology*; D011247:Pregnancy; D011994:Recombinant Proteins / Q000008:administration & dosage; D013915:Thrombasthenia / Q000150:complications*; D016896:Treatment Outcome
chemical_list: D011994:Recombinant Proteins; D015942:Factor VIIa