TMA's

Question 1

New-onset hemolysis in the setting of renal failure should always prompt consideration of?

Answer 1

TTP (thrombotic thrombocytopenic purpura)

Question 2

TTP commonly presents with?

Answer 2

presents with nonspecific abdominal pain, fevers, changes in mentation, thrombocytopenia, petechiae and purpura, and hemolytic anemia

Question 3

Pathophysiology of TTP?

Answer 3

The ultimate underlying pathophysiology is reduced activity of von Willebrand factor-cleaving protease ADAMTS13. While this can be a hereditary deficiency, when presenting in adults, this is often due to the de novo production of an inhibitor. If untreated, TTP is almost universally fatal

Question 4

Treatment of TTP?

Answer 4

Even with appropriate treatment, up to 10% of cases may fail to improve. Plasma exchange, developed in the 1980s, results in removal of the inhibitor and improvement in platelet counts, and reversal of the underlying pathophysiology. The concomitant administration of glucocorticoids and rituximab results in the reversal of the underlying autoimmune process.