Surgical procedures in patients with Glanzmann's thrombasthenia: case series and literature review

Blood Coagul Fibrinolysis. 2017 Mar;28(2):171-175. doi: 10.1097/MBC.0000000000000524.

Abstract

Glanzmann's thrombasthenia is a rare platelet function disorder with an autosomal recessive pattern of inheritance. Achieving haemostasis in such patients who undergo surgical procedures always poses a significant challenge. Herein we report six cases of Glanzmann's thrombasthenia, who underwent nine surgeries under the cover of platelet-rich concentrates with or without recombinant activated factor VII . Of these, five were major surgeries such as thyroidectomy, laparotomy, Hartmann's procedure, reversal of Hartmann's procedure and a complete dental extraction. All five procedures were successfully done without any major bleeding. The major cost incurred in these procedures is due to the large number of blood products used and recombinant activated factor VII if used.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Factor VIIa / administration & dosage
  • Factor VIIa / therapeutic use*
  • Female
  • Humans
  • Male
  • Recombinant Proteins / administration & dosage
  • Recombinant Proteins / therapeutic use
  • Thrombasthenia / drug therapy
  • Thrombasthenia / surgery*
  • Young Adult

Substances

  • Recombinant Proteins
  • recombinant FVIIa
  • Factor VIIa