Managing Acute Hemorrhage in Glanzmann Thrombasthenia in the Emergency Department : A Two-Case Report

• Rim Karray

  CHU Habib Bourguiba Sfax

• Fedi Ben Ali

• Racem Mnif

• Hela Ben Jmeaa

• Hana Kessentini

• Imed Frikha

• Olfa Chakroun

• Noureddine Rekik

Abstract : 

Background: Glanzmann Thrombasthenia (GT) is a rare inherited platelet function disorder caused by a quantitative or qualitative deficiency of the glycoprotein IIb/IIIa complex, leading to defective platelet aggregation. Although typically associated with mucocutaneous bleeding, life-threatening hemorrhagic complications may occur and pose significant challenges in emergency settings.

Cases presentation: We report two cases of acute hemorrhage in patients with GT managed in the emergency department. The first case involved a 32-year-old man presenting with acute dyspnea due to a massive spontaneous left hemothorax. Despite prompt resuscitation, blood and platelet transfusions, antifibrinolytic therapy, and emergency chest drainage, the patient developed respiratory failure and subsequently died from septic shock. The second case concerned a 19-year-old man admitted after facial trauma complicated by persistent epistaxis refractory to local measures and tranexamic acid. Hemostasis was successfully achieved after platelet transfusion.

Conclusion: These cases highlight the wide clinical spectrum of hemorrhagic emergencies in GT and the limitations of standard coagulation tests, which are often normal. Management relies on rapid assessment, early antifibrinolytic therapy, and platelet transfusion, with recombinant activated factor VII as a valuable option in refractory or allo-immunized patients.

Emergency physicians should be aware of this rare condition, as timely multidisciplinary management is crucial to reduce morbidity and mortality.

1. Fedi Ben Ali

   Emergency Department, Habib Bourguiba University Hospital, Sfax, Tunisia.

2. Racem Mnif

   Emergency Department, Habib Bourguiba University Hospital, Sfax, Tunisia.

3. Hela Ben Jmeaa

   Cardiovascular and Thoracic Surgery Department, Habib Bourguiba University Hospital, Sfax, Tunisia

4. Hana Kessentini

   Emergency Department, Habib Bourguiba University Hospital, Sfax, Tunisia.

5. Imed Frikha

     Cardiovascular and Thoracic Surgery Department, Habib Bourguiba University Hospital, Sfax, Tunisia.

6. Olfa Chakroun

    Emergency Department, Habib Bourguiba University Hospital, Sfax, Tunisia.

7. Noureddine Rekik

   Emergency Department, Habib Bourguiba University Hospital, Sfax, Tunisia.

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