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

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

Type/Severity	Criteria
Laboratory values change^a	The occurrence of a change in ≥ 2 laboratory values^b: • 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 TMA^d	Clinical signs and symptoms considered definitely related to aHUS, including: • Thrombosis • Seizure • Reduction in renal function • Proteinuria (new or worse^c and > 1+ or > 30 mg/dL) • Hematuria (new or worse^c and > 50 RBCs/HPF) • Increased hemolytic anemia • Biopsy-proven TMA • Other (eg, extrarenal TMA manifestations, including confusion, cardiovascular abnormalities, pericarditis, gastrointestinal symptoms/diarrhea)
Intervention^d	The patient required PE/PI, dialysis, blood transfusions, or renal transplant due to a TMA manifestation

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
^aAs determined by changes in laboratory parameters with ongoing follow-up
^bMeasurements were required to be confirmed by a second measurement ≥ 28 days apart with no interruption
^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
^dAs determined at the discretion of the investigator

ISSN: 1471-2369