Bernard-Soulier syndrome
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Bernard-Soulier syndrome
Hematological system
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Bernard-Soulier syndrome
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Bernard-Soulier syndrome
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| 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. 419, 434, 730
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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.
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