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Table 5 Proposed working diagnostic criteria for TTP-like syndrome

From: TTP-like syndrome: novel concept and molecular pathogenesis of endotheliopathy-associated vascular microthrombotic disease

1. Thrombocytopenia and MAHA/aMAHA.
2. Underling critical illness due to such conditions as.
Pathogen (bacterial; viral; fungal; rickettsial; parasitic)
Polytrauma (chest and lung; bone; skull/brain)
Pregnancy (preeclampsia; abruptio placenta; amniotic fluid embolism)
Cancer (breast; stomach; lung)
Surgery (heart; bowel; uterus; bone)
Transplant (liver; kidney; bone marrow)
Disease (autoimmune vascular disease; malignant hypertension)
Drug (cyclosporin; mitomycin C)
Toxin (venom; ricin; Shiga toxin)
3. Negative antibody against ADAMTS13.
4. Mild to moderately decreased activity of ADAMTS13 (20–70% of normal).
5. One or more organ phenotype dysfunction syndromes such as.
Myocardial infarction.
Acute fulminant hepatic failure.
Acute adrenal insufficiency.
Non-occlusive mesenteric ischemia.
Hepato-renal syndrome.
Cardio-pulmonary syndrome.
Tissue gangrene.
Peripheral digit ischemic syndrome.
  1. + Encephalopathy and ARF are common in both TTP and TTP-like syndrome
  2. ARDS acute respiratory distress syndrome, TTP thrombotic thrombocytopenic purpura