eTable 2. ADAMTS13 activity >10%: atypical hemolytic uremic syndrome, secondary hemolytic uremic syndrome / thrombotic microangiopathy.

Indication for continued plasmapheresis should be rigorously examined							Discontinue plasmapheresis
Very rare HUS form (e.g. for children <1 year)	Complement-mediated HUS		Infections	Autoimmune diseases	Drug-induced TMA		Disseminated cancer	Malignant hypertension
DGKE mutations	Atypical hemolytic uremic syndrome	TA-TMA	HIV, CMV, influenza H1N1, parvovirus B19	SLE, catastrophic antiphospholipid syndrome (CAPS), ANCA pos. vasculitis, MPGN	Dose dependent, endothelial cell damage	Dose independent, antibody mediated	Prostate, breast, or lung cancer with bone marrow carcinomatosis, intravascular tumor cells	Known hypertension, echocardiography: hypertensive heart disease
Plasminogen mutations	Elavated sC5b-9, anti-CFH Ab	PBSCT, organ transplantation
Cobalamin defect	Mutations: C3, factor H, factor I, factor B, thrombomodulin			Rarely: ADAMTS13 reduced	Mitomycin C, gemcitabine, bevacicumab, calcineurin-inhibitors	Chinidin, oxaliplatin, ticlopidin
No standard therapy is available	Eculizumab	Discontinue calcineurin inhibitors	Antiviral therapy (e.g.. HAART)	CAPS: anticoagulation, plasmapheresis	Discontinue medication		Treatment of the underlying disease	Antihypertensive therapy
Cobalamin defect: Vitamin B12 substitution	Anti-CFH positive: additional plasmapheresis	(Eculizumab off-label use)	If ADAMTS13 antibodies are detected: therapy as for aTTP	SLE: immunosuppression	If ADAMTS13 antibodies are detected: therapy as for aTTP

ADAMTS13: a disintegrin and metalloprotease with thrombospondin type 1 motif, member 13; ANCA: antineutrophil cytoplasmic antibodies; aTTP: immune-mediated, acquired thrombotic-thrombocytopenic purpura; CAPS: catastrophic antiphospholipid syndrome;

CFH: complement factor H; CMV: cytomegalovirus; DGKE: diacylglycerol kinase; HAART: highly active antiretroviral therapy; HIV: human immunodeficiency virus; HUS: hemolytic uremic syndrome; MPGN: membranoproliferative glomerulonephritis;

PBSCT: peripheral blood stem cell transplantation; SLE: systemic lupus erythematosus; TA-TMA: transplant-associated thrombotic microangiopathy; TMA: thrombotic microangiopathy