Perioperative haemostatic management of Glanzmann thrombasthenia for abdominal surgery

Blood Coagul Fibrinolysis. 2009 Jul;20(5):371-3. doi: 10.1097/MBC.0b013e32832b280e.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antifibrinolytic Agents / administration & dosage
  • Antifibrinolytic Agents / therapeutic use*
  • Blood Coagulation Tests
  • Blood Loss, Surgical
  • Blood Transfusion, Autologous
  • Blood Volume
  • Combined Modality Therapy
  • Crystalloid Solutions
  • Elective Surgical Procedures
  • Factor VIIa / administration & dosage
  • Factor VIIa / therapeutic use*
  • Female
  • Fibrinolysin / administration & dosage
  • Fibrinolysin / therapeutic use*
  • Heart Septal Defects, Ventricular / complications
  • Heart Valve Diseases / complications
  • Humans
  • Hypertension, Pulmonary / complications
  • Hysterectomy*
  • Isotonic Solutions / administration & dosage
  • Perioperative Care / methods
  • Platelet Transfusion*
  • Preanesthetic Medication*
  • Recombinant Proteins / administration & dosage
  • Recombinant Proteins / therapeutic use
  • Thrombasthenia / complications
  • Thrombasthenia / drug therapy
  • Thrombasthenia / therapy*
  • Tranexamic Acid / administration & dosage
  • Tranexamic Acid / therapeutic use*

Substances

  • Antifibrinolytic Agents
  • Crystalloid Solutions
  • Isotonic Solutions
  • Recombinant Proteins
  • Tranexamic Acid
  • recombinant FVIIa
  • Factor VIIa
  • Fibrinolysin