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The patient in this vignette presents with mucocutaneous petechiae, bleeding gums, and an inability to bind fibrinogen suggestive of Glanzmann thrombasthenia. In this condition, the only abnormal serological finding may be an increased bleeding time. Glanzmann thrombasthenia is an autosomal recessive bleeding disorder caused by a defect in the GpIIb/IIIa receptor. This receptor lies on the platelet surface and functions to promote platelet aggregation by binding to fibrinogen. Key laboratory findings in Glanzmann thrombasthenia include a normal platelet count with normal platelet morphology, normal PT, normal PTT, and increased bleeding time. Figure A is a table depicting the possible laboratory changes in this patient; see explanations in Incorrect Answers section for a further description. Incorrect Answers: Answer 1: Decreased platelet count, elevated PT, elevated PTT, and elevated bleeding time is characteristic of disseminated intravascular coagulation (DIC). DIC is a systemic coagulopathy characterized by pathologic coagulation, microthrombi formation, and depletion of coagulation factors. Answer 2: Decreased platelet count, normal PT, normal PTT, and elevated bleeding time is characteristic of multiple hematologic disorders including thrombotic thrombocytopenic purpura (TTP), idiopathic thrombocytopenic purpura (ITP), and Bernard-Soulier disease. Answer 4: Normal platelet count, normal PT, elevated PTT, and elevated bleeding time is characteristic of von Willebrand disease. This condition is characterized by an intrinsic pathway coagulation defect and impaired platelet adherence. Answer 5: Normal platelet count, normal PT, elevated PTT, and normal bleeding time is characteristic of a patient with a defect in the intrinsic coagulation pathway. The most common clinical cause of this scenario would be anticoagulation treatment with heparin. Bullet Summary: Glanzmann thrombasthenia is characterized by an increased bleeding time with an otherwise normal platelet count, platelet morphology, PT, and PTT.
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