Table 2.

TMA Harmonization Panel Consensus Recommended Diagnostic Criteria, Modified Jodele Criteria

Biopsy-proven disease (kidney or GI) or
Clinical criteria: must meet ≥4 of the following 7 criteria within 14 days at 2 consecutive time points
Anemia*	Defined as one of the following: 1. Failure to achieve transfusion independence for pRBCs despite evidence of neutrophil engraftment 2. Hemoglobin decline from patient’s baseline by 1 g/dL 3. New onset of transfusion dependence Rule out other causes of anemia, such as AIHA and PRCA
Thrombocytopenia*	Defined as one of the following: 1. Failure to achieve platelet engraftment 2. Higher than expected platelet transfusion needs 3. Refractoriness to platelet transfusions 4. 50% reduction or greater in baseline platelet count after full platelet engraftment
Elevated LDH	>ULN for age
Schistocytes	Present
Hypertension	>99th percentile for age (<18 yr), or systolic BP ≥140 mmHg or diastolic BP ≥90 mmHg (≥18 yr)
Elevated sC5b-9	≥ULN
Proteinuria	≥1 mg/mg rUPCR

pRBCs indicates packed red blood cells; AIHA, autoimmune hemolytic anemia; PRCA, pure red cell aplasia; BP, blood pressure. upper limit of normal; ULN, random urine protein to creatinine ratio (rUPCR)

^*Indicates clarification from published Jodele et al. criteria¹.