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/mm 3 Leukocyte count 9,000/mm 3 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?

Bleeding from the sphenopalatine artery

Bernard-Soulier syndrome

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Bernard-Soulier syndrome

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NOTES NOTES PLATELET DISORDERS GENERALLY, WHAT ARE THEY? PATHOLOGY & CAUSES ▪ Platelet dysfunction, impaired hemostasis, bleeding RISK FACTORS DIAGNOSIS LAB RESULTS ▪ Complete blood count (CBC) ▪ Clotting studies (e.g. bleeding time) ▪ Platelet function tests ▪ Children of parents who are close relatives (consanguineous) COMPLICATIONS ▪ Mild to severe hemorrhage SIGNS & SYMPTOMS ▪ Mucocutaneous bleeding ▪ Excessive bruising/bleeding after minor trauma ▪ Immediate, excessive bleeding with invasive procedures TREATMENT ▪ Manage spontaneous/trauma-related bleeding episodes MEDICATIONS ▪ ▪ ▪ ▪ Anti-ﬁbrinolytic therapy Platelet transfusions Avoid antiplatelet medications Corticosteroids, immunosuppressants BERNARD–SOULIER SYNDROME (BSS) osms.it/bernard-soulier-syndrome PATHOLOGY & CAUSES ▪ Rare, inherited clotting disorder; mild thrombocytopenia, macrothrombocytopenia (giant platelets), platelet dysfunction, bleeding CAUSES ▪ Autosomal recessive inheritance pattern 444 OSMOSIS.ORG ▪ Platelets lack essential glycoprotein Ib-IX-V complex (GPIb) → impaired hemostasis → bleeding RISK FACTORS ▪ Prevalence in individuals of Mediterranean descent ▪ Children of parents who are close relatives (consanguineous)

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Bernard-Soulier syndrome

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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.

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