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Know the Signs of Glanzmann's Thrombasthenia.

Glanzmann's thrombasthenia (GT) is a rare bleeding disorder that requires careful diagnosis.1 For a clinical resource on a diagnostic algorithm for GT, reach out to your Novo Nordisk Representative.

What is Glanzmann’s thrombasthenia?

Glanzmann’s thrombasthenia is a bleeding disorder that’s characterized by impaired platelet function. Patients may experience symptoms ranging from minimal bruising to potentially fatal hemorrhaging.2,3

4-year-old patient experiencing severe epistaxis.4,a

aThis is an actual patient case.

A correct diagnosis is crucial.

Most patients with Glanzmann’s thrombasthenia present with spontaneous and moderate bleeds. However, 26% of bleeds can be severe.5,b

bBased on data from Glanzmann’s Thrombasthenia Registry (GTR).

Glanzmann’s thrombasthenia diagnoses are very rare.

Glanzmann’s thrombasthenia affects ~1 in 1 million people.2 Patients typically begin showing symptoms in childhood.6

Walk through the diagnostic steps.

Your local Novo Nordisk Representative can share a diagnostic algorithm and management guidelines for Glanzmann’s thrombasthenia.

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Selected Important Safety Information

WARNING: THROMBOSIS

  • Serious arterial and venous thrombotic events following administration of NovoSeven® RT have been reported
  • Discuss the risks and explain the signs and symptoms of thrombotic and thromboembolic events to patients who will receive NovoSeven® RT
  • Monitor patients for signs or symptoms of activation of the coagulation system and for thrombosis

Warnings and Precautions

  • Serious arterial and venous thrombotic events have been reported in clinical trials and postmarketing surveillance
  • Patients with congenital hemophilia receiving concomitant treatment with aPCCs (activated prothrombin complex concentrates), older patients particularly with acquired hemophilia and receiving other hemostatic agents, and patients with a history of cardiac and vascular disease may have an increased risk of developing thrombotic events

Indications and Usage

NovoSeven® RT (coagulation Factor VIIa, recombinant) is a coagulation factor indicated for:

  • Treatment of bleeding episodes and perioperative management in adults and children with hemophilia A or B with inhibitors, congenital Factor VII (FVII) deficiency, and Glanzmann’s thrombasthenia with refractoriness to platelet transfusions, with or without antibodies to platelets
  • Treatment of bleeding episodes and perioperative management in adults with acquired hemophilia

Important Safety Information

WARNING: THROMBOSIS

  • Serious arterial and venous thrombotic events following administration of NovoSeven® RT have been reported
  • Discuss the risks and explain the signs and symptoms of thrombotic and thromboembolic events to patients who will receive NovoSeven® RT
  • Monitor patients for signs or symptoms of activation of the coagulation system and for thrombosis

Warnings and Precautions

  • Serious arterial and venous thrombotic events have been reported in clinical trials and postmarketing surveillance
  • Patients with congenital hemophilia receiving concomitant treatment with aPCCs (activated prothrombin complex concentrates), older patients particularly with acquired hemophilia and receiving other hemostatic agents, and patients with a history of cardiac and vascular disease may have an increased risk of developing thrombotic events
  • Hypersensitivity reactions, including anaphylaxis, can occur with NovoSeven® RT. Patients with a known hypersensitivity to mouse, hamster, or bovine proteins may be at a higher risk of hypersensitivity reactions. Discontinue infusion and administer appropriate treatment when hypersensitivity reactions occur
  • Factor VII deficient patients should be monitored for prothrombin time (PT) and factor VII coagulant activity (FVII:C). If FVII:C fails to reach the expected level, or PT is not corrected, or bleeding is not controlled after treatment with the recommended doses, antibody formation may be suspected and analysis for antibodies should be performed
  • Laboratory coagulation parameters (PT/INR, aPTT, FVII:C) have shown no direct correlation to achieving hemostasis

Adverse Reactions

  • The most common and serious adverse reactions in clinical trials are thrombotic events. Thrombotic adverse reactions following the administration of NovoSeven® RT in clinical trials occurred in 4% of patients with acquired hemophilia and 0.2% of bleeding episodes in patients with congenital hemophilia

Drug Interactions

  • Thrombosis may occur if NovoSeven® RT is administered concomitantly with Coagulation Factor XIII

Please click here for Prescribing Information including boxed Warning.

References

  1. Solh T, Botsford A, Solh M. Glanzmann’s thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options. J Blood Med. 2015;6:219-227.
  2. Di Minno G, Coppola A, Di Minno MND, et al. Glanzmann’s thrombasthenia (defective platelet integrin alpha(IIb)-beta(3)): proposals for management between evidence and open issues. Thromb Haemost. 2009;102(6):1157-1164.
  3. Nurden AT, Pillois X, Nurden P. Understanding the genetic basis of Glanzmann thrombasthenia: implications for treatment. Exp Rev Hematol. 2012;5(5):487-503.
  4. Recht M, Chitlur M, Lam D, et al. Epistaxis as a common presenting symptom of Glanzmann’s thrombasthenia, a rare qualitative platelet disorder: illustrative case examples. Case Rep Emerg Med. 2017:8796425.
  5. Di Minno G, Zotz RB, d’Oiron R, et al. Haematologica. 2015;100(8):1031-1037.
  6. Poon MC, Di Minno G, d’Oiron R, et al. New insights into the treatment of Glanzmann thrombasthenia. Transfus Med Rev. 2016;30(2):92-99.