Diagnostic criteria for TA-TMA
| Clinical/laboratory finding . | BMT-CTN criteria32 . | IWG-EBMT criteria5 . | Overall TMA criteria33 . | Refined TMA criteria34 . |
|---|---|---|---|---|
| Normal coagulation assays* | Yes | Yes | Yes | NR |
| Schistocytosis | >2 per HPF on PS | >8 per HPF on PS | >2 per HPF on PS | Yes |
| Increase in serum LDH | Yes | Yes | Yes | Yes |
| Thrombocytopenia† | NR | Yes | Yes | Yes |
| Anemia | NR | Yes | Yes | Yes |
| Decrease in serum haptoglobin | NR | Yes | Yes | NR |
| Negative Coombs test | Yes | NR | Yes | NR |
| Renal and/or CNS involvement | Yes | NR | NR | NR |
| Proteinuria | NR | NR | NR | Random urinalysis proteinuria concentration ≥30 mg/dL‡ |
| Hypertension | NR | NR | NR | 3-18 y: BP at 95th percentile value for age, sex, and height; >18 y: BP ≥140/90 mm Hg |
| Terminal complement assay | NR | NR | NR | Elevated plasma concentration of sC5b-9‡ |
| Clinical/laboratory finding . | BMT-CTN criteria32 . | IWG-EBMT criteria5 . | Overall TMA criteria33 . | Refined TMA criteria34 . |
|---|---|---|---|---|
| Normal coagulation assays* | Yes | Yes | Yes | NR |
| Schistocytosis | >2 per HPF on PS | >8 per HPF on PS | >2 per HPF on PS | Yes |
| Increase in serum LDH | Yes | Yes | Yes | Yes |
| Thrombocytopenia† | NR | Yes | Yes | Yes |
| Anemia | NR | Yes | Yes | Yes |
| Decrease in serum haptoglobin | NR | Yes | Yes | NR |
| Negative Coombs test | Yes | NR | Yes | NR |
| Renal and/or CNS involvement | Yes | NR | NR | NR |
| Proteinuria | NR | NR | NR | Random urinalysis proteinuria concentration ≥30 mg/dL‡ |
| Hypertension | NR | NR | NR | 3-18 y: BP at 95th percentile value for age, sex, and height; >18 y: BP ≥140/90 mm Hg |
| Terminal complement assay | NR | NR | NR | Elevated plasma concentration of sC5b-9‡ |
Doubling of serum creatinine from baseline (baseline creatinine before hydration and conditioning) or 50% decrease in creatinine clearance from baseline.
BP, blood pressure; BMT-CTN, Blood and Marrow Transplant Clinical Trials Network; CNS, central nervous system; HPF, high-power field; IWG, International Working Group; LDH, lactose dehydrogenase; PS, peripheral smear.
Coagulation assays included prothrombin time and activated partial thromboplastin time.
De novo, prolonged, or progressive thrombocytopenia (platelet count 50 × 109/L or 50% reduction from previous counts).
Parameters of severity of TMA, according to Jodele et al.34