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2020 ISTH TTP Pocket Guideline with GPS

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5 Table 3. PLASMIC score or French score predicts the likelihood of severe ADAMTS13 deficiency in a suspected TTP Parameters French Score PLASMIC Score Platelet count <30 × 10 9 /L (+1) <30 × 10 9 /L (+1) Serum creatinine level <2.26 mg/dL (+1) <2.0 mg/dL (+1) Hemolysis Indirect bilirubin >2 mg/dL * +1 or reticulocyte count >2.5 % or undetectable haptoglobin No active cancer in previous year * +1 No history of solid organ or SCT * +1 INR <1.5 * +1 MCV <90 f L NA +1 Likelihood of severe deficiency of ADAMTS13 activity (<10 %) 0: 2% 0–4: 0–4% 1: 70% 6: 5–24% 2: 94% 6–7: 62–82% Each item is associated with one point (+1). *French score considered patients with thrombotic microangiopathy (TMA) that included hemolysis and schistocytes in their definition and assumed that there was no history or clinical evidence for associated cancer, transplantation or disseminated intravascular coagulation. erefore, these items were intrinsic to the scoring system. e table is adopted from Joly B.S. Expert Rev Hematol. 2019;12:383-95. ➤ In settings of no reasonable access to plasma ADAMTS13 testing, the panel suggests that caplacizumab not be used, regardless of the pretest probability of immune TTP. (C-L). • TPE and steroids, plus rituximab may still be offered in the scenario.

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