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Updated: Jul 9 2020

Bernard-Soulier Disease

  • Overview
https://upload.medbullets.com/topic/111017/images/platelet aggregation.jpg
  • Snapshot
    https://upload.medbullets.com/topic/111017/images/giant-platelet-syndrome-6431_0.jpg
    • A 3-week-old boy is brought to the pediatrician to undergo a circumcision. Despite the procedure being uncomplicated, the bleeding has not subsided after a few hours. Complete blood count is significant for a platelet count of 85,000/mm3. Peripheral blood smear is shown. Platelet aggregation studies show no response to ristocetin. This was not corrected with the addition of normal plasma. With the addition of ADP, normal aggregation was observed.
  • Differential
    • Glanzmann thrombasthenia
    • Idiopathic thrombocytopenic purpura (ITP)
    • von Willebrand disease
  • Prognosis, Prevention, and Complications
    • Varying bleeding severity throughout life
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Hematology | Bernard-Soulier Disease
  • Hematology
  • - Bernard-Soulier Disease
11:38 min
2/1/2022
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4.0
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