血小板无力症
维基百科，自由的百科全书
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| ICD-10 | D69.1 |
| ICD-O: | {{{ICDO}}} |
| ICD-9 | 287.1 |
| OMIM | {{{OMIM}}} |
| MedlinePlus | {{{MedlinePlus}}} |
| eMedicine | {{{eMedicineSubj}}}/{{{eMedicineTopic}}} |
| DiseasesDB | {{{DiseasesDB}}} |
血小板无力症 是一种极其罕见的血液疾病，原因是血小板缺乏糖蛋白IIb/IIIa受体(GPIIB/IIIA), 所以纤维蛋白原很难交联，致使出血时间显著延长。
目录 |
[编辑] 病理生理学
The GpIIb/IIIa is an adhesion receptor and is expressed in thrombocytes. This receptor is activated when the thrombocyte is stimulated by 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 trombocytes via fibrinogen.
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.
The understanding of its pathophysiology lead to the development of GpIIb/IIIa inhibitors, a class of powerful antiplatelet agents.[1]
[编辑] 病因学
血小板无力症 can be inherited in an autosomal recessive manner[1] or acquired as an autoimmune disorder.[2]
[编辑] 临床特点
Characteristically, there is increased mucosal bleeding:
- 流鼻血
- 经血过多
- increased bleeding post-operatively.
The bleeding tendency is variable but may be severe.
Patients present with moderate bleeding and normal platelet morphology. Aggregation of platelets occurs in response to ristocetin, but not to other agonists such as ADP, thrombin, collagen or adrenalin.
[编辑] 参考文献
- ^ 1.0 1.1 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. Free Full Text.
- ↑ 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.
[编辑] 参见
- 凝血因子
- 血液疾病
