Thrombotic microangiopathy after renal transplantation: Current insights in de novo and recurrent disease

Table 4 Genotype-phenotype correlations in atypical hemolytic uremic syndrome (data refer to the period before introduction of eculizumab)

Gene	Risk of death or ESRD at onset or first yr	Risk ofrecurrence	Risk of death or ESRDafter 3-5 yr	Risk of recurrencein allograft
CFH or CFH-CFHR1/3 hybrid genes	50%-70%	50%	75%	75%-90%
CFI	50%	10%-30%	50%-60%	45%-80%
MCP single	0%-6%	70%-90%	6%-38%	< 20%
MCP combined1	30%-40%	50%	50%	50%-60%
C3	60%	50%	75%	40%-70%
CFB	50%	100%	75%	100%
THBD	50%	30%	54%	?
Anti-FH	30%-40%	40%-60%	35%-60%	Depends on antibody titers

¹Combined with CFH or CFI or C3 mutations. Adapted from: Goodship et al[58]. CFB: Complement factor B gene; CFH: Complement factor H gene; CFHR: Complement factor H-related genes; CFI: Complement factor I gene; FH: Factor H protein; THBD: Thrombomodulin gene.