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Bernard-Soulier syndrome

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Hematological system

Pathology

Anemias
Heme synthesis disorders
Coagulation disorders
Platelet disorders
Mixed platelet and coagulation disorders
Thrombosis syndromes (hypercoagulability)
Lymphomas
Leukemias
Leukemoid reaction
Dysplastic and proliferative disorders
Plasma cell dyscrasias
Hematological system pathology review

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Bernard-Soulier syndrome

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High Yield Notes
3 pages
Flashcards

Bernard-Soulier syndrome

10 flashcards
Questions

USMLE® Step 1 style questions USMLE

2 questions
Preview

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.  
 

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 
*Reference Range: 2-7 minutes  

Which of the following conditions is the patient at greatest risk of developing?   

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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.