Perioperative haemostatic management of Glanzmann thrombasthenia for abdominal surgery.
Glanzmann thrombasthenia is a rare congenital platelet disorder characterized by spontaneous mucocutaneous bleeding and severe bleeding complications during major surgery. This report centres on the perioperative haemostatic management of a patient with Glanzmann thrombasthenia undergoing elective major abdominal surgery. The treatment regimen was based mainly on recombinant activated factor VII, fibrinogen, and factor XIII, reducing platelet transfusion to a minimum. No red blood cell transfusions were needed perioperatively. For haemostatic monitoring, routine laboratory tests were sufficient.
Lison
Susanne
S
Department of Anesthesiology, Ludwig Maximilians University of Munich, Munich, Germany. susanne.lison@med.uni-muenchen.de
Spannagl
Michael
M
Heindl
Bernhard
B
D000328:Adult; D000933:Antifibrinolytic Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D001780:Blood Coagulation Tests; D016063:Blood Loss, Surgical; D001804:Blood Transfusion, Autologous; D001810:Blood Volume; D003131:Combined Modality Therapy; D000077324:Crystalloid Solutions; D017558:Elective Surgical Procedures; D015942:Factor VIIa / Q000008:administration & dosage / Q000627:therapeutic use*; D005260:Female; D005341:Fibrinolysin / Q000008:administration & dosage / Q000627:therapeutic use*; D006345:Heart Septal Defects, Ventricular / Q000150:complications; D006349:Heart Valve Diseases / Q000150:complications; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications; D007044:Hysterectomy; D007552:Isotonic Solutions / Q000008:administration & dosage; D019990:Perioperative Care / Q000379:methods; D017713:Platelet Transfusion; D011229:Preanesthetic Medication; D011994:Recombinant Proteins / Q000008:administration & dosage / Q000627:therapeutic use; D013915:Thrombasthenia / Q000150:complications / Q000188:drug therapy / Q000628:therapy*; D014148:Tranexamic Acid / Q000008:administration & dosage / Q000627:therapeutic use*
D000933:Antifibrinolytic Agents; D000077324:Crystalloid Solutions; D007552:Isotonic Solutions; D011994:Recombinant Proteins; D014148:Tranexamic Acid; C103587:recombinant FVIIa; D015942:Factor VIIa; D005341:Fibrinolysin