Thrombotic Thrombocytopenic Purpura Sequelae

An ASH ’25 summary by Dr. Wolfgang Miesbach: iTTP remission ≠ cure–the vascular injury persists. TTP as a cardiovascular disease equivalent. I just finished Dr. Senthil Sukumar’s session on long-term iTTP management. This completely changed how I think about these patients, as illustrated:

• 28.6% of survivors → major cardiovascular events
• 50% → silent cerebral infarcts (vs 16.6% controls)
• Cardiovascular complications 10–20 years earlier
• 80% depression–35% post traumatic stress disorder
• >60% measurable neurocognitive impairment
• 71% moderate-severe headaches
• 20% can’t work due to complications

The game changer: endothelial damage that doesn’t heal. Patients are not “fine” after remission. The endothelium stays injured. Stress cardiac MRI shows reduced perfusion and impaired coronary vasodilation—the heart can’t increase blood flow under stress, even when ADAMTS13 levels are normal.

The actionable part:

• ADAMTS13 ≤70% = 27.6% stroke rate
• ADAMTS13 >70% = ZERO ischemic strokes

  As a consequence, treat iTTP as CVD equivalent:

• Comprehensive cardiovascular risk factor optimization (lipids, A1C, smoking, BP, weight, activity)
• Baseline mood + neurocognitive screening for ALL survivors
• Neuropsych testing every 1–2 years
• Multidisciplinary teams (neuro, psych, cardio)