Glanzmann's Thrombasthenia - Treatment

Treatment

Therapy involves both preventive measures and treatment of specific bleeding episodes.

  • Dental hygiene lessens gingival bleeding
  • Avoidance of antiplatelet agents such as aspirin and other anti-inflammatory drugs (NSAIDs) such as ibuprofen and naproxen, and anticoagulants
  • Iron or folate supplementation may be necessary if excessive or prolonged bleeding has caused anemia
  • Hepatitis B vaccine
  • Antifibrinolytic drugs such as tranexamic acid or ε-aminocaproic acid (Amicar)
  • Desmopressin (DDAVP) does not normalize the bleeding time in Glanzmann's thrombasthenia but anecdotally improves hemostasis
  • Hormonal contraceptives to control excessive menstrual bleeding
  • Topical agents such as gelfoam, fibrin sealants, polyethylene glycol polymers, custom dental splints
  • Platelet transfusions (only if bleeding is severe; risk of platelet alloimmunization)
  • Recombinant factor VIIa
  • Hematopoietic stem cell transplantation (HSCT) for severe recurrent hemorrhages

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