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Pathology - Thrombotic Thrombocytopenic Purpura (TTP)
This condition is medically identified by the presence of five specific symptoms: fever, low platelet count, kidney failure, abnormal breakdown of red blood cells with fragmented cells called schistocytes, and abnormalities in the nervous system. The idiopathic form of this thrombotic microangiopathy commonly presents with a deficit of the vWF cleaving protease ADAMTS-13, caused by an autoimmune response. This leads to the presence of excessively large multimers of vWF, which in turn promote excessive platelet aggregation. Thrombocytopenia occurs due to the excessive formation of microvascular thrombi. Petechiae and purpura occur as a consequence of thrombocytopenia. Cerebral microvascular occlusion can result in various neurological symptoms such as headache, seizures, localized neurological impairments, or alterations in mental status. Renal microinfarcts result in the development of acute renal failure, which is characterized by an increase in serum creatinine levels. Erythrocytes undergo shearing when they come into contact with microthrombi, leading to the development of hemolytic anemia. This condition causes the formation of schistocytes and jaundice. Plasma exchange is the preferred treatment and platelet transfusion can actually exacerbate the production of microthrombi.
This condition is medically identified by the presence of five specific symptoms: fever, low platelet count, kidney failure, abnormal breakdown of red blood cells with fragmented cells called schistocytes, and abnormalities in the nervous system. The idiopathic form of this thrombotic microangiopathy commonly presents with a deficit of the vWF cleaving protease ADAMTS-13, caused by an autoimmune response. This leads to the presence of excessively large multimers of vWF, which in turn promote excessive platelet aggregation. Thrombocytopenia occurs due to the excessive formation of microvascular thrombi. Petechiae and purpura occur as a consequence of thrombocytopenia. Cerebral microvascular occlusion can result in various neurological symptoms such as headache, seizures, localized neurological impairments, or alterations in mental status. Renal microinfarcts result in the development of acute renal failure, which is characterized by an increase in serum creatinine levels. Erythrocytes undergo shearing when they come into contact with microthrombi, leading to the development of hemolytic anemia. This condition causes the formation of schistocytes and jaundice. Plasma exchange is the preferred treatment and platelet transfusion can actually exacerbate the production of microthrombi.
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