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| {{SI}} | | {{Glanzmann's thrombasthenia}} |
| {{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.
| | {{SK}} Glanzmann thrombasthenia; Glanzmann's disease |
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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>
| | ==[[Glanzmann's thrombasthenia overview|Overview]]== |
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| ==Etiology== | | ==[[Glanzmann's thrombasthenia historical perspective|Historical Perspective]]== |
| 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== | | ==[[Glanzmann's thrombasthenia classification|Classification]]== |
| 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]].
| | ==[[Glanzmann's thrombasthenia pathophysiology|Pathophysiology]]== |
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| ==Eponym== | | ==[[Glanzmann's thrombasthenia causes|Causes]]== |
| 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== | | ==[[Glanzmann's thrombasthenia differential diagnosis|Differentiating Glanzmann's thrombasthenia from Other Diseases]]== |
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| ===Gallery=== | | ==[[Glanzmann's thrombasthenia epidemiology and demographics|Epidemiology and Demographics]]== |
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| ====Extremities==== | | ==[[Glanzmann's thrombasthenia risk factors|Risk Factors]]== |
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| <gallery>
| | ==[[Glanzmann's thrombasthenia screening|Screening]]== |
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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>
| | ==[[Glanzmann's thrombasthenia natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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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>
| | ==Diagnosis== |
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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>
| | [[Glanzmann's thrombasthenia history and symptoms|History and Symptoms]] |
| | | [[Glanzmann's thrombasthenia physical examination|Physical Examination]] |
| | | [[Glanzmann's thrombasthenia laboratory findings|Laboratory Findings]] |
| | | [[Glanzmann's thrombasthenia chest x ray|Chest X Ray]] |
| | | [[Glanzmann's thrombasthenia CT|CT]] |
| | | [[Glanzmann's thrombasthenia other imaging findings|Other Imaging Findings]] |
| | | [[Glanzmann's thrombasthenia other diagnostic studies|Other Diagnostic Studies]] |
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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>
| | ==Treatment== |
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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>
| | [[Glanzmann's thrombasthenia medical therapy|Medical Therapy]] |
| | | [[Glanzmann's thrombasthenia primary prevention|Primary Prevention]] |
| | | [[Glanzmann's thrombasthenia secondary prevention|Secondary Prevention]] |
| | | [[Glanzmann's thrombasthenia cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] |
| | | [[Glanzmann's thrombasthenia future or investigational therapies|Future or Investigational Therapies]] |
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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>
| | ==Case Studies== |
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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>
| | [[Glanzmann's thrombasthenia case study one|Case #1]] |
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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>
| | [[Category:Disease]] |
| | | [[Category:Hematology]] |
| </gallery>
| | [[Category:Primary care]] |
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| ==References==
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| {{reflist|2}}
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