Which condition is characterized by renal dysfunction and requires treatment with plasmapheresis?

Thrombotic Thrombocytopenic Purpura (TTP) is a rare but serious condition characterized by a combination of microangiopathic hemolytic anemia, thrombocytopenia, fluctuating neurological symptoms, fever, and renal dysfunction. The underlying pathophysiology involves the deficiency of the von Willebrand factor-cleaving protease, ADAMTS13, which leads to the formation of large von Willebrand factor multimers that promote excessive platelet aggregation and can cause the occlusion of small blood vessels.

Renal dysfunction in TTP occurs due to microvascular obstruction and damage to the renal glomeruli, as well as due to associated hemolysis. This can lead to elevated creatinine levels and signs of acute kidney injury.

Plasmapheresis is the primary treatment for TTP, as it helps to remove the autoantibodies against ADAMTS13 and replenishes the deficient enzyme. This intervention is critical to restoring normal blood flow and function in the microvasculature, thereby improving renal and overall organ function.

The other conditions listed do not characteristically involve the need for plasmapheresis as part of their standard treatment protocols. For example, hemophilia is primarily treated through factor replacement therapy