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Table 1 Definition of TMA manifestation (any one or more listed criteria) [11]

From: Outcomes in patients with atypical hemolytic uremic syndrome treated with eculizumab in a long-term observational study

Type/Severity Criteria
Laboratory values changea The occurrence of a change in ≥ 2 laboratory valuesb:
• Platelet count decrease ≥ 25%c and < LLN
• Increase in SCr ≥ 25%c and > ULN
• Increase in LDH ≥ 25%c and > ULN
Clinical signs and symptoms of TMAd Clinical signs and symptoms considered definitely related to aHUS, including:
• Thrombosis
• Seizure
• Reduction in renal function
• Proteinuria (new or worsec and > 1+ or > 30 mg/dL)
• Hematuria (new or worsec and > 50 RBCs/HPF)
• Increased hemolytic anemia
• Biopsy-proven TMA
• Other (eg, extrarenal TMA manifestations, including confusion, cardiovascular abnormalities, pericarditis, gastrointestinal symptoms/diarrhea)
Interventiond The patient required PE/PI, dialysis, blood transfusions, or renal transplant due to a TMA manifestation
  1. aHUS atypical hemolytic uremic syndrome; HPF high-powered field; LDH lactate dehydrogenase; LLN lower limit of normal; PE/PI plasma exchange/plasma infusion; RBCs red blood cells; SCr serum creatinine; TMA thrombotic microangiopathy; ULN upper limit of normal
  2. aAs determined by changes in laboratory parameters with ongoing follow-up
  3. bMeasurements were required to be confirmed by a second measurement ≥ 28 days apart with no interruption
  4. cDuring each on period, compared with the last laboratory value during the preceding off period. During each off period, compared with the last value during the preceding on period
  5. dAs determined at the discretion of the investigator