**Core Concept:**
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. It is caused by a deficiency in ADAMTS13, an enzyme responsible for cleaving von Willebrand factor (VWF).
**Why the Correct Answer is Right:**
The correct answer is "Option C" that states TTP is caused by autoimmune disease. While TTP can indeed be caused by various factors, the most common cause is a deficiency in ADAMTS13, which cleaves von Willebrand factor (VWF) and prevents the formation of large, pathogenic VWF multimers that cause endothelial damage and microthrombi formation. Autoimmune diseases are less common causes of TTP.
**Why Each Wrong Option is Incorrect:**
A. Thrombocytopenia is a core feature of TTP, not a cause.
B. Von Willebrand disease is a separate disorder characterized by abnormal VWF function, not the cause of TTP.
D. Autoimmune disease is a less common cause of TTP, making it incorrect in this context.
**Clinical Pearl / High-Yield Fact:**
In cases where a patient presents with the classical TTP syndrome (microangiopathic hemolytic anemia, thrombocytopenia, renal failure, neurological symptoms, fever, and decreased ADAMTS13 activity), it is essential to promptly recognize and initiate plasma exchange therapy, which is the standard treatment for TTP. Establishing the correct diagnosis is crucial, as early treatment can significantly improve patient outcomes.
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