GpIIb/IIIa is essential to blood coagulation since the activated receptor has the ability to bind fibrinogen (as well as von Willebrand factor, fibronectin, and vitronectin), which is required for fibrinogen-dependent platelet-platelet interaction (aggregation). Understanding of the role of GpIIb/IIIa in Glanzmanns thrombasthenia led to the development of GpIIb/IIIa inhibitors, a class of powerful antiplatelet agents. Diagnosis
Light transmission aggregometry is widely accepted as the gold standard diagnostic tool for assessing platelet function, and a result of absent aggregation with any agonist except ristocetin is highly specific for Glanzmanns thrombasthenia. Following is a table comparing its result with other platelet aggregation disorders:

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 Glanzmanns 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, AryoSeven or NovoSeven FDA approved this drug for the treatment of the disease in July 2014. Hematopoietic stem cell transplantation (HSCT) for severe recurrent hemorrhages

Eponym
It is named after Eduard Glanzmann (1887-1959), the Swiss pediatrician who originally described it.
History
The subsequent studies, following Eduard Glanzmanns  description of hemorrhagic symptoms and "weak platelets" demonstrated that these patients have prolonged bleeding times and their platelets failed to aggregate in response to activation. In the mid-1970s, Nurden and Caen and Phillips and colleagues discovered that thrombasthenic platelets are deficient in integrins  αIIbβ3. See also
Platelet
Coagulation
Bernard-Soulier syndrome

References


== External links ==