JAMA Clinical Reviews Immune Thrombotic Thrombocytopenic Purpera (iTTP)
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May 19, 2025 Join Allyson M. Pishko, an Assistant Professor of Medicine in Hematology Oncology at the University of Pennsylvania, as she dives into the complexities of immune thrombotic thrombocytopenic purpura (iTTP). She explains the critical nature of early diagnosis and treatment options like plasma exchange. The conversation highlights the innovative role of caplicizumab in enhancing patient care, the importance of monitoring treatment responses, and the risks associated with catheter placement, shedding light on this rare but life-threatening disorder.
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Understanding Thrombotic Microangiopathies
- Thrombotic microangiopathies involve clots in small blood vessels causing microangiopathic hemolytic anemia and low platelets.
- These clots can cause life-threatening organ damage, like heart attacks and strokes, needing urgent treatment.
Diagnosing Immune TTP
- Diagnose iTTP by confirming microangiopathic hemolytic anemia and thrombocytopenia, then exclude more common causes.
- Confirm iTTP with severe ADAMTS13 enzyme deficiency under 10% to clinch the diagnosis.
Role of ADAMTS13 in iTTP
- The ADAMTS13 enzyme cleaves large von Willebrand factor multimers to prevent excessive clotting.
- Deficiency leads to ultra-large multimers causing platelets to bind and occlude small vessels, triggering iTTP.