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Glanzmann's thrombasthenia
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Glanzmann's thrombasthenia : ウィキペディア英語版
Glanzmann's thrombasthenia

Glanzmann's thrombasthenia is an abnormality of the platelets. It is an extremely rare coagulopathy (bleeding disorder due to a blood abnormality), in which the platelets contain defective or low levels of glycoprotein IIb/IIIa (GpIIb/IIIa), which is a receptor for fibrinogen. As a result, no fibrinogen bridging of platelets to other platelets can occur, and the bleeding time is significantly prolonged.
==Signs and symptoms==
Characteristically, there is increased mucosal bleeding:〔
* menorrhagia
* easy bruising
* epistaxis
* gingival bleeding
* gastrointestinal bleeding
* postpartum bleeding
* increased bleeding post-operatively.
The bleeding tendency is variable but may be severe. Hemarthrosis, particularly spontaneous, is very rare, in contrast to the hemophilias.
Platelet numbers and morphology are normal. Platelet aggregation is normal with ristocetin, but impaired with other agonists such as ADP, thrombin, collagen or epinephrine.

抄文引用元・出典: フリー百科事典『 ウィキペディア(Wikipedia)
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