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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.

Pancreatitis.

Myocardial infarction.

ARDS.

Acute fulminant hepatic failure.

Acute adrenal insufficiency.

Rhabdomyolysis.

Non-occlusive mesenteric ischemia.

Hepato-renal syndrome.

Hepatic-encephalopathy.

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