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Primary Hemostasis

Inherited or Acquired Disorders

Inherited disorders affecting primary hemostasis include disorders of the vascular system and disorders of platelet function. Disorders of the vascular system affect the involvement of the blood vessels in hemostasis and may be due to abnormality in or damage to the endothelial lining or subendothelial structures. Such disorders may be inherited, such as hereditary telangiectasia and Marfan’s syndrome, or acquired, such as scurvy or Cushing syndrome.1

Disorders of platelet function affect the ability of platelets to form the primary hemostatic plug. Such disorders are categorized as either quantitative or qualitative. In quantitative platelet disorders, platelet count is either too low (thrombocytopenia) or too high (thrombocytosis). Thrombocytopenia may arise from increased destruction of platelets due to immune (e.g., idiopathic thrombocytopenic purpura) and nonimmune mechanisms or decreased platelet production. Thrombocytosis may result from a defect in the control mechanism for platelet production.1
 


Qualitative platelet disorders involve inherited or acquired abnormalities in any phase of platelet function, resulting in a defect in the primary hemostatic plug. Inherited abnormalities may affect platelet adhesion (e.g., von Willebrand disease [vWD]), platelet aggregation (e.g., Glanzmann Thrombasthenia, or platelet secretion (e.g., Hermansky-Pudlak syndrome).1

Acquired disorders of platelet function may result from the effect of certain drugs on normal platelet function. Antiplatelet agents (e.g., aspirin, ADP antagonists, glycoprotein IIb/IIIa inhibitors) are commonly responsible for abnormal platelet function. While the effect of these drugs in patients with normal hemostatic function is minimal, patients with abnormal hemostatic function face elevated risk of bleeding symptoms.1,2

To learn more how to cost-effectively and efficiently identify these disorders, click here.


References

 

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