Learning the signs is the first step toward recognizing Glanzmann’s thrombasthenia
Learning the signs is the first step toward recognizing Glanzmann’s thrombasthenia
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Glanzmann’s thrombasthenia (GT) is a rare bleeding disorder characterized by impaired platelet function. Patients may experience symptoms ranging from minimal bruising to potentially fatal hemorrhages. Glanzmann’s thrombasthenia shares symptoms with other bleeding disorders, making diagnosis difficult.1-4
GT: a rare bleeding disorder that’s challenging to diagnose
Extremely
rare
Only approximately 1 in 1 million people are affected by GT1
Difficult to
diagnose
Similar symptoms as other acquired platelet disorders and vWD3,4
Diverse patient population
GT affects both males and females, often without a diagnosed family history5
vWD=von Willebrand disease
GT is caused by defects in the GPIIb/IIIa receptor on platelets6
- GPIIb/IIIa, a receptor on the platelet surface, mediates platelet aggregation6
- GPIIb/IIIa is decreased or dysfunctional in patients with GT6
- As a result, platelet plugs do not form effectively during vessel injury, leading to lengthy episodes of bleeding6
When do GT patients experience their first bleed symptoms?
aBased on data from Glanzmann’s Thrombasthenia Registry (GTR).
GT patients’ bleeds can be severe
Most patients with Glanzmann’s thrombasthenia present with spontaneous and moderate bleeds. However, ~26% of bleeds in GT patients are severe.7,b
Severity of bleeding episodes
Types of bleeding episodes
Types of bleeding episodes
Learn how to spot the signs of Glanzmann’s thrombasthenia
Review the common signs and symptoms of Glanzmann’s thrombasthenia and see how patients typically present with this rare bleeding disorder.
References:
- 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.
- Nurden AT, Pillois X, Nurden P. Understanding the genetic basis of Glanzmann thrombasthenia: implications for treatment. Exp Rev Hematol. 2012;5(5):487-503
- Solh T, Botsford A, Solh M. Glanzmann’s thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options. J Blood Med. 2015;6:219-227.
- Lambert MP. What to do when you suspect an inherited platelet disorder. Hematology Am Soc Hematol Educ Program. 2011;2011:377-383.
- Sharathkumar AA, Shapiro AD. Platelet Function Disorders. 2nd ed. Montréal, Québec, Canada: World Federation of Hemophilia; 2008.
- 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.
- Di Minno G, Zotz RB, d’Oiron R, et al. The international, prospective Glanzmann Thrombasthenia Registry: treatment modalities and outcomes of non-surgical bleeding episodes in patients with Glanzmann thrombasthenia. Haematologica. 2015;100(8):1031-1037.