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| __NOTOC__
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| {{SI}}
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| {{CMG}}; {{AE}} {{JH}}.
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| ==Overview==
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| '''Glanzmann's thrombasthenia''' is an extremely rare [[Blood diseases|disorder]] of the [[blood]], in which the [[platelet]]s lack [[glycoprotein IIb/IIIa]]. Hence, no [[fibrinogen]] bridging can occur, and bleeding time is significantly prolonged.
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| ==Pathophysiology==
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| The [[GpIIb/IIIa]] is an adhesion receptor and is expressed in [[thrombocytes]]. This receptor is activated when the thrombocyte is stimulated by [[Adenosine diphosphate|ADP]], [[epinephrine]], [[collagen]] and [[thrombin]]. The [[GpIIb/IIIa]] [[integrin]] is essential to the [[blood coagulation]] since it has the ability to bind [[fibrinogen]], the [[von Willebrand factor]], [[fibronectin]] and [[vitronectin]]. This enables the platelet to be activated by contact with the collagen-von Willebrand-complex that is exposed when the endothelial blood vessel lining is damaged and then aggregate with other [[thrombocytes]] via [[fibrinogen]].
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| Patients suffering from Glanzmann's thrombasthenia thus have platelets less able to adhere to each other and to the underlying tissue of damaged blood vessels.
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| The understanding of its pathophysiology led to the development of [[GpIIb/IIIa inhibitors]], a class of powerful [[antiplatelet agent]]s.<ref name=seligsohn>Seligsohn U. Glanzmann thrombasthenia: a model disease which paved the way to powerful therapeutic agents. Pathophysiol Haemost Thromb. 2002 Sep-Dec;32(5-6):216-7. PMID 13679645. [http://content.karger.com/ProdukteDB/produkte.asp?Aktion=ShowPDF&ArtikelNr=73569&ProduktNr=224034&Ausgabe=229381&filename=73569.pdf Free Full Text].</ref>
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| ==Etiology==
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| Glanzmann's thrombasthenia can be inherited in an [[autosomal]] [[recessive]] manner<ref name=seligsohn/> or acquired as an [[autoimmune disorder]].<ref>Tholouli E, Hay CR, O'Gorman P, Makris M. Acquired Glanzmann's thrombasthenia without thrombocytopenia: a severe acquired autoimmune bleeding disorder. Br J Haematol. 2004 Oct;127(2):209-13. PMID 15461628.</ref>
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| ==Clinical Features==
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| Characteristically, there is increased mucosal bleeding:
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| *[[epistaxis]]
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| *[[menorrhagia]]
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| *increased bleeding post-operatively.
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| The bleeding tendency is variable but may be severe.
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| Patients present with moderate [[bleeding]] and normal [[platelet]] morphology. [[Aggregation]] of [[platelet]]s occurs in response to [[ristocetin]], but not to other [[agonist]]s such as [[Adenosine diphosphate|ADP]], [[thrombin]], [[collagen]] or [[adrenalin]].
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| ==Eponym==
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| It is named for Eduard Glanzmann.<ref>{{WhoNamedIt|synd|1289}}</ref><ref>W. E. Glanzmann. Hereditäre hämorrhägische Thrombasthenie. Ein Beitrag zur Pathologie der Blutplättchen. Jahrbuch für Kinderheilkunde, 1918; 88: 1-42, 113-141.</ref>
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| ==Physical examination==
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| ===Gallery===
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| ====Extremities====
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| <gallery>
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| Image: Glanzmann's_trombastenia_01.jpeg| Glanzmann’s trombastenia. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=12 With permission from Dermatology Atlas.]''<ref name="www.atlasdermatologico.com.br">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=164>
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| Image: Glanzmann's_trombastenia_02.jpeg| Glanzmann’s trombastenia. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=12 With permission from Dermatology Atlas.]''<ref name="www.atlasdermatologico.com.br">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=164>
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| Image: Glanzmann's_trombastenia_03.jpeg| Glanzmann’s trombastenia. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=12 With permission from Dermatology Atlas.]''<ref name="www.atlasdermatologico.com.br">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=164>
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| Image: Glanzmann's_trombastenia_04.jpeg| Glanzmann’s trombastenia. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=12 With permission from Dermatology Atlas.]''<ref name="www.atlasdermatologico.com.br">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=164>
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| Image: Glanzmann's_trombastenia_05.jpeg| Glanzmann’s trombastenia. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=12 With permission from Dermatology Atlas.]''<ref name="www.atlasdermatologico.com.br">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=164>
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| Image: Glanzmann's_trombastenia_06.jpeg| Glanzmann’s trombastenia. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=12 With permission from Dermatology Atlas.]''<ref name="www.atlasdermatologico.com.br">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=164>
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| Image: Glanzmann's_trombastenia_07.jpeg| Glanzmann’s trombastenia. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=12 With permission from Dermatology Atlas.]''<ref name="www.atlasdermatologico.com.br">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=164>
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| Image: Glanzmann's_trombastenia_08.jpeg| Glanzmann’s trombastenia. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=12 With permission from Dermatology Atlas.]''<ref name="www.atlasdermatologico.com.br">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=164>
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| </gallery>
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| ==References==
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| {{reflist|2}}
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