Recognize TMA
- Schistocytes + hemolysis markers + thrombocytopenia
- Assess neurologic, cardiac and renal ischemic injury
- The historic pentad is not required
Recognize a thrombotic microangiopathy, estimate the probability of immune TTP, obtain ADAMTS13 before plasma therapy when feasible, and start time-critical TTP treatment without waiting for the result when suspicion is high.
Thrombocytopenia + microangiopathic hemolysis: treat high-probability immune TTP before confirmatory testing returns
High-probability immune TTP is a treatment-before-confirmation emergency: plasma exchange should not wait for ADAMTS13 turnaround.
Confirm microangiopathic hemolysis (schistocytes, elevated LDH/indirect bilirubin, low haptoglobin, reticulocytosis) with thrombocytopenia and ischemic organ injury. Renal, neurologic and cardiac involvement can vary; the historic pentad is neither required nor expected.
Use clinical judgment plus a validated pretest tool such as PLASMIC when appropriate. Severe thrombocytopenia with MAHA and relatively preserved coagulation studies raises concern for TTP; markedly abnormal PT/aPTT/fibrinogen favors DIC, while heparin exposure/thrombosis may suggest HIT.
Send ADAMTS13 activity/inhibitor before plasma exposure whenever this can be done without delaying treatment. Severe ADAMTS13 deficiency (<10%) supports TTP, but the result often returns too late to guide the first lifesaving decision.
Current ISTH guidance for acute immune TTP treatment, including plasma exchange, immunosuppression and caplacizumab.
Evidence status
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From emergent massive-hemorrhage protocol activation and early balanced component resuscitation to physiologic correction, targeted factor/anticoagulant reversal, and definitive source control.
From assessing hemodynamic stability to correcting reversible contributors to coagulopathy.
Urgent maternal stabilization, left uterine displacement, syndromic differentiation, and multidisciplinary escalation.
Recognition of thrombotic microangiopathy, PLASMIC risk, ADAMTS13 testing, immediate plasma exchange, steroids, caplacizumab, and ICU complications.
Tissue-factor-driven coagulation activation, antithrombin/protein C depletion, PAI-1-associated fibrinolytic suppression, ISTH 2025 DIC assessment, comparison with TTP/HUS/HIT, and targeted replacement.
PF4-heparin antibody Fc-gamma-RIIa platelet activation, the 4Ts score, ELISA OD values vs Serotonin Release Assay (SRA), and direct thrombin inhibitor protocols.