Bernard-Soulier syndrome
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Bernard-Soulier syndrome
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USMLE® Step 1 style questions USMLE
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A 9-year-old boy is brought to the emergency department by his parents due to prolonged bleeding following a tooth extraction earlier in the day. Past medical history is noncontributory. Temperature is 37.5°C (99.5°F), pulse is 88/min, respirations are 14/min, and blood pressure is 112/62 mmHg. Physical exam shows gingival bleeding and petechiae. Laboratory testing is obtained, and the results are shown below.
*Reference Range: 2-7 minutes
Which of the following conditions is the patient at greatest risk of developing?
Laboratory value | Result |
Hematologic | |
Hemoglobin | 12 g/dL |
Hematocrit | 40% |
Platelet count | 95,000/mm3 |
Leukocyte count | 9,000/mm3 |
Coagulation studies | |
Prothrombin time (PT) | 12 seconds |
Activated partial thromboplastin time (aPTT) | 29 seconds |
Bleeding time* | 15 minutes |
Which of the following conditions is the patient at greatest risk of developing?
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Bernard-Soulier syndrome p. 417, 432, 733
Summary
Bernard-Soulier syndrome (BSS) is a rare congenital bleeding disorder characterized by thrombocytopenia, unusually giant platelets, and prolonged bleeding time. Patients with BSS typically have prolonged or severe bleeding after minor trauma or surgery and may also develop epistaxis, hematomas, and petechiae. The severity of the condition can vary from mild to life-threatening.