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Disorders of Primary Hemostasis: USMLE Step 1 Pathology

Disorders of primary hemostasis involve abnormalities in the initial steps of blood clot formation, particularly platelet adhesion, activation, and aggregation. These disorders can result from inherited or acquired conditions that affect platelet function or number, leading to impaired formation of the platelet plug. 💉 Von Willebrand disease (vWD) is the most common inherited disorder of primary hemostasis, characterized by deficiency or dysfunction of von Willebrand factor (vWF), a protein essential for platelet adhesion. Other inherited disorders include Bernard-Soulier syndrome and Glanzmann thrombasthenia, which involve defects in platelet glycoproteins crucial for platelet-platelet and platelet-vessel wall interactions. Acquired disorders of primary hemostasis include thrombocytopenia, where there is a reduced number of circulating platelets, and medication-induced platelet dysfunction, commonly seen with aspirin or nonsteroidal anti-inflammatory drugs (NSAIDs). Clinical manifestations of disorders of primary hemostasis include mucocutaneous bleeding, such as epistaxis, petechiae, and easy bruising, as well as prolonged bleeding after minor trauma or surgery. Diagnosis involves laboratory tests such as platelet count, bleeding time, and specific assays for von Willebrand factor and platelet function. Treatment strategies vary depending on the underlying cause and may include platelet transfusions, desmopressin (DDAVP) for vWD, and medications to stimulate platelet production. Understanding the pathophysiology, clinical features, and management of disorders of primary hemostasis is essential for medical students preparing for the USMLE Step 1 exam.

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