Which of the following is a type of thrombotic microangiopathic purpura that typically includes features such as renal involvement and neurologic symptoms?

Which of the following is a type of thrombotic microangiopathic purpura that typically includes features such as renal involvement and neurologic symptoms?

• A. Hemolytic-uremic syndrome
• B. Thrombotic thrombocytopenia purpura (TTP)
• C. Thrombotic microangiopathic purpura
• D. DIC

Answer: (B)

Thrombotic thrombocytopenic purpura is a thrombotic microangiopathy in which small-vessel platelet-rich clots produce microangiopathic hemolytic anemia and thrombocytopenia, with neurologic symptoms and often renal involvement. The key driver is severe deficiency of ADAMTS13, the enzyme that cleaves ultra-large von Willebrand factor multimers. Without this cleavage, ULVWF multimers cause widespread platelet aggregation in small vessels, leading to red blood cell fragmentation as they pass through the obstructions (schistocytes), low platelets, and the clinical features. Neuro symptoms arise from microthrombi in cerebral vessels, while kidney involvement occurs from renal microvasculopathy. This pattern helps distinguish TTP from HUS, which is typically more kidney-focused and often post-diarrheal, and from DIC, where coagulation tests are abnormal due to widespread consumption of clotting factors. Urgent plasma exchange is the mainstay of treatment to remove antibodies against ADAMTS13 and replenish the enzyme.

Thrombotic thrombocytopenic purpura is a thrombotic microangiopathy in which small-vessel platelet-rich clots produce microangiopathic hemolytic anemia and thrombocytopenia, with neurologic symptoms and often renal involvement. The key driver is severe deficiency of ADAMTS13, the enzyme that cleaves ultra-large von Willebrand factor multimers. Without this cleavage, ULVWF multimers cause widespread platelet aggregation in small vessels, leading to red blood cell fragmentation as they pass through the obstructions (schistocytes), low platelets, and the clinical features. Neuro symptoms arise from microthrombi in cerebral vessels, while kidney involvement occurs from renal microvasculopathy. This pattern helps distinguish TTP from HUS, which is typically more kidney-focused and often post-diarrheal, and from DIC, where coagulation tests are abnormal due to widespread consumption of clotting factors. Urgent plasma exchange is the mainstay of treatment to remove antibodies against ADAMTS13 and replenish the enzyme.