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Decreased platelet aggregation on peripheral blood smear
6%
10/155
Immune production of anti-platelet antibodies
30%
47/155
Increased prothrombin time and partial thromboplastin time
8%
12/155
Increased serum von Willebrand factor multimers
48%
74/155
Large platelets on peripheral blood smear
2%
3/155
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This patient with fever, headache, fatigue, conjunctival pallor, petechiae, an increased bleeding time, and decreased platelets most likely has thrombotic thrombocytopenic purpura, which would present with increased serum von Willebrand factor multimers. Thrombotic thrombocytopenic purpura is caused by a deficiency or inhibition of the ADAMTS13 metalloproteinase, which normally functions to separate von Willebrand factor (vWF) multimers into monomers. In this disorder, the persistence of vWF multimers leads to the formation of microvascular platelet thrombosis and systemic thrombocytopenia since the platelets are consumed in this process. Furthermore, shearing of erythrocytes that travel through these thrombosed vessels leads to a hemolytic anemia. Together, this disease then presents with a classic pentad of microangiopathic hemolytic anemia, acute renal failure, thrombocytopenia, fever, and neurologic abnormalities, though most patients do not have all the signs. Incorrect Answers: Answer 1: Decreased platelet aggregation on peripheral blood smear would be seen in Glanzmann thrombasthenia; however, this disease would not present with conjunctival pallor since it does not cause anemia. Answer 2: Immune production of anti-platelet antibodies would be seen in idiopathic thrombocytopenia; however, this disease would not present with conjunctival pallor since it does not cause anemia. Answer 3: Increased prothrombin time and partial thromboplastin time would be seen in disseminated intravascular coagulation; however, this patient has relatively stable vital signs. Furthermore, 5 months of fatigue suggests a longer-term etiology of anemia rather than acute bleeding or sepsis. Answer 5: Large platelets on peripheral blood smear would be seen in Bernard-Soulier disease; however, this disease would not present with conjunctival pallor since it does not cause anemia. Bullet Summary: Thrombotic thrombocytopenic purpura will present with increased serum von Willebrand factor multimers.
2.4
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