Table 2.

Evaluations to perform after confirmation of TA-TMA.

Evaluation	Notes
Complement: C3, sC5b-9, CH50	C3 level is typically low (representing consumption) Elevated sC5b9 is a risk-factor for poor outcome Baseline CH50 useful if eculizumab is to be administered
Reticulocyte count (trend) and Immature Platelet Fraction (trend)	Useful to determine if marrow production is able to keep up with destruction
GVHD biomarkers: ST2, REG3-alpha, ± HGF, ± Elafin	Useful to determine if GVHD is driving the TA-TMA
Chimerism (T-cell)	May be useful to determine if sub-clinical GVHD is driving the TA-TMA
CRP (trend)	May be useful to determine if uncontrolled inflammation is driving the TA-TMA
Viral PCRs (CMV, Adenovirus, HHV-6, BK) and Galactomannan	Useful to determine if uncontrolled infection is driving the TA-TMA
Antibodies to CFH & Class II HLA	Marker of antibody-mediated TA-TMA
Electrocardiogram and Echocardiogram	Request specific evaluation of RV pressure and Tricuspid Regurgitation Velocity
Consider genetic panel (complement)	Must be done on buccal swab or banked DNA (if allogeneic HCT)

GVHD, graft-vs.-host disease; ST2, suppressor of tumorigenicity 2; REG3, regenerating islet-derived 3; HGF, hepatocyte growth factor; CRP, C-reactive protein; CMV, cytomegalovirus; HHV-6, human herpes virus-6; CFH, complement factor H; HLA, human leukocyte antigen.