Thrombotic Thrombocytopenic Purpura: A Rare Case Presenting with Splenic Infarction
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چکیده
Thrombotic Thrombocytopenic Purpura (TTP) is a hypercoagulable state, in which platelets aggregate and clot in the microvasculature. The majority of cases can be linked to ADAMST13 deficiencies, a protein responsible for cleaving VonWillibrand’s Factor (vWF). Hypo functional ADAMST13 results in un-mitigated vWF activity, leading to excessive platelet aggregation. Increased clotting leads to decreased circulating platelets, hemolysis and anemia. Microvascular infarctions cause non-blanching purpura, renal damage, and cerebral ischemia resulting in a myriad of Central Nervous System manifestations from altered mental status to seizures. While some cases are idiopathic, 10% of TTP diagnoses are associated with sepsis [1] or malignancy [2] as well as enterocolitis, especially from E. coli [3].
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