Table 5.
Proposed working diagnostic criteria for TTP-like syndrome
| 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. |
+ Encephalopathy and ARF are common in both TTP and TTP-like syndrome
ARDS acute respiratory distress syndrome, TTP thrombotic thrombocytopenic purpura