title: Glanzmann's thrombasthenia proposed optimal management during surgery and delivery.
abstract: Glanzmann's thrombasthenia (GT) is an autosomal recessive disorder of platelet function. Conventional management is by platelet transfusion, given before invasive interventions. Alloimmunization resulting in platelet refractoriness and an unpredictable response to platelet infusion have provided particular management difficulties in the past. More recently recombinant (r)VIIa (Novoseven) has a valuable role in the treatment of platelet function disorders. Treatment of a patient with GT during two pregnancies and spinal surgery is reported. An algorithm is presented to provide a structured and consistent approach to treatment.
authors.affiliation: Centre for Haemostasis and Thrombosis, The Haemophilia Reference Centre, St. Thomas' Hospital, London, UK.
authors.forename: Julie-Anne
authors.initials: JA
authors.lastname: Bell
authors.forename: Geoffrey F
authors.initials: GF
authors.lastname: Savidge
mesh_terms: D000063:Accidents, Traffic; D000328:Adult; D000465:Algorithms; D016063:Blood Loss, Surgical; D019090:Case Management; D036861:Delivery, Obstetric; D017586:Diskectomy; D004388:Dura Mater / Q000293:injuries; D005167:Factor VII / Q000627:therapeutic use*; D015942:Factor VIIa; D005260:Female; D015788:HLA-A1 Antigen / Q000276:immunology; D006801:Humans; D007114:Immunization; D007518:Isoantibodies / Q000276:immunology; D007751:Labor, Induced; D008159:Lumbar Vertebrae / Q000601:surgery*; D010923:Placenta Previa / Q000150:complications; D019039:Platelet Glycoprotein GPIIb-IIIa Complex / Q000276:immunology; D017713:Platelet Transfusion; D006473:Postpartum Hemorrhage / Q000209:etiology / Q000517:prevention & control; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000628:therapy*; D011994:Recombinant Proteins / Q000627:therapeutic use*; D013915:Thrombasthenia / Q000628:therapy*; D014592:Uterine Hemorrhage / Q000209:etiology
chemical_list: D015788:HLA-A1 Antigen; D007518:Isoantibodies; D019039:Platelet Glycoprotein GPIIb-IIIa Complex; D011994:Recombinant Proteins; D005167:Factor VII; C103587:recombinant FVIIa; D015942:Factor VIIa