Two case reports of Glanzmann thrombasthenia with intracranial hemorrhage and a review of the literature.

Surg Neurol Int

Department of Neurosurgery, King Abdulaziz Medical City - National Guard Health Affairs, Riyadh, Saudi Arabia.

Published: December 2023

AI Article Synopsis

  • Glanzmann's thrombasthenia (GT) is a rare genetic disorder leading to poor platelet function, causing varying degrees of bleeding, and even intracranial bleeding is uncommon.
  • The study details two patient cases: a 9-year-old girl with a spontaneous epidural hematoma and a 20-year-old man with a subarachnoid hemorrhage following a car accident, both of whom received conservative management.
  • The findings suggest that patients with GT experiencing intracranial hemorrhage can be effectively managed through careful observation and treatment involving platelet transfusions and certain medications without immediate surgery.

Article Abstract

Background: Glanzmann's thrombasthenia (GT) is a rare autosomal recessive disorder characterized by impaired platelet function. Symptoms range from mild to life-threatening bleeding. However, it is extremely rare for a patient to have intracranial bleeding. This study presents two cases of GT: one with a spontaneous epidural hematoma (EDH) and the other with a subarachnoid hemorrhage due to traumatic causes. The discussion that follows then derives relevant supporting insights through a review of the literature.

Case Description: Case Report 1: A 9-year-old girl with a known case of GT presented to an emergency department with a severe headache but no other complaints or history of trauma. The physical examination was normal. Computed tomography (CT) head without contrast revealed multiple EDHs with no midline shift. She received factor VII, tranexamic acid, and platelets transfusion and was admitted to the intensive care unit to be managed conservatively. After a month, a CT head follow-up showed complete resolution of all hematomas. Case Report 2: A 20-year-old male with a known case of GT was brought to the hospital with a history of loss of consciousness for several minutes after a road traffic accident. He suffered from a headache on regaining consciousness and received analgesia. CT head showed diffuse subarachnoid hemorrhage. He was managed with factor VII, tranexamic acid, and platelets transfusion and was admitted to an intermediate care unit for close observation.

Conclusion: In a GT patient with intracranial hemorrhage, conservative management with close clinical observation and platelet transfusion in combination with recombinant activated factor VII and/or antifibrinolytics can be safely conducted.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10783678PMC
http://dx.doi.org/10.25259/SNI_680_2023DOI Listing

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