Table 1.

Classification of thrombotic microangiopathies

Primary TMA: hereditary

aHUS with complement gene mutation

(CFH; CFI; CFB; C3; CD46; CFHR1 hybrid)

TTP with ADAMTS13 mutation

MMACHC TMA

DGKE TMA

Primary TMA: hereditary

aHUS with complement autoantibodies

(anti-FH; anti-FI)

TTP with ADAMTS13 autoantibody

Secondary TMAs

TMA with glomerular disease

(FSGS; IgAN, C3G/MPGN, MN, AAV)

Malignancy associated TMA

Drug induced TMA

Direct toxicity (interferon B; bevacizumab)

Immune mediated damage (e.g., quinine)

TMA with autoimmune conditions

(SLE, SRC, CAPS)

De novo TMA after solid organ transplant

HELLP

Infection associated TMA

STEC-HUS

Pneumococcal HUS

HIV associated aHUS

Other

AAV ANCA (anti-neutrophil cytoplasmic antibody) associated vasculitis; ADAMTS13 a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13; aHUS atypical hemolytic uremic syndrome; C3G C3 glomerulopathy; CAPS catastrophic antiphospholipid syndrome; MMACHC Methylmalonic aciduria and homocystinuria, cblC type; DGKE gene encoding diacylglycerol kinase Ɛ; FH factor H; FI factor I, FSGS focal segmental glomerulosclerosis; HELLP syndrome of hemolysis, elevated liver enzymes, and low platelets; HIV human immunodeficiency virus; HUS hemolytic uraemic syndrome; IgAN IgA nephropathy; MN membranous nephropathy; MPGN membranoproliferative glomerulonephritis; SLE systemic lupus erythematosus; SRC scleroderma renal crisis; STEC, shiga toxin-producing Escherichia coli; TMA thrombotic microangiopathy; TTP thrombotic thrombocytopenic purpura