1) A 9-year-old boy is seen for prolonged bleeding after tooth extraction. He has a history of easy bruising and epistaxis. PE: gingival bleed and skin petechiae and bruises of various ages. Labs: Prolonged bleeding time, Low platelet count. Workup shows deficient platelet Gp1 receptors. Diagnosis? a) Bernard-Soulier disease b) Glanzmann thrombasthenia c) Immune thrombocytopenic purpura d) Thrombotic thrombocytopenic purpura 2) A 9-year-old boy is seen for prolonged bleeding after tooth extraction. He has a history of easy bruising and epistaxis. PE: gingival bleed and skin petechiae and bruises of various ages. Labs: Prolonged bleeding time, Low platelet count.  Workup shows deficient platelet Gp1 receptors. What aspect of blood clot formation is altered?  a) Binding to subendothelial collagen b) Fibrin mess formation c) Platelet adhesion d) Platelet aggregation 3) A 9-year-old boy is seen for prolonged bleeding after tooth extraction. He has a history of easy bruising and epistaxis. PE: gingival bleed and skin petechiae and bruises of various ages. Labs: Prolonged bleeding time, Low platelet count. Workup shows deficient platelet Gp1 receptors. What additional lab test would be abnormal??  a) D-dimer b) Prothrombin time c) Partial Thromboplastin Time d) Ristocetin platelet aggregation study 4) A 6-year-old boy is seen for a laceration on the arm that won't stop bleeding. He has a history of frequent epistaxis. Labs: Prolonged bleeding time; normal platelet count, PT, PTT. Ristocetin platelet aggregation study is normal. Administration of desmopression does not improve the bleeding time. Diagnosis? a) Bernard-Soulier disease b) Glanzmann thrombasthenia c) Immune thrombocytopenic purpura d) von Willebrand disease 5) A 26-year-old female has a history of SLE. She complains of dizziness, fatigue, menorrhagia, and rashes on her legs. PE: pallor, petechiae on lower extremities. Labs: low platelet count, Hb, Hct. Normal WBC, haptoglobin, total bilirubin. Diagnosis? a) Bernard-Soulier disease b) Glanzmann thrombasthenia c) Immune thrombocytopenic purpura d) Thrombotic thrombocytopenic purpura 6) A 26-year-old female is brought to emergency dept in confusion. She had no significant past medical history. PE: Fever, petechiae and ecchymosis on arms, trunk, and legs. Labs: low platelet count, Hb, Hct. Normal WBC, haptoglobin, total bilirubin. Elevated BUN, creatinine. Diagnosis? a) Bernard-Soulier disease b) Glanzmann thrombasthenia c) Immune thrombocytopenic purpura d) Thrombotic thrombocytopenic purpura 7) A 26-year-old female is brought to emergency dept in confusion. She had no significant past medical history. PE: Fever, petechiae and ecchymosis on arms, trunk, and legs. Labs: low platelet count, Hb, Hct. Normal WBC, haptoglobin, total bilirubin. Elevated BUN, creatinine. Which of following is abnormal in this patient?? a) Heme synthesis b) Hemoglobin synthesis c) Platelet receptor function d) von Willebrand factor multimer degradation

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