Table 5.

Treatment

(i) Supportive measures only

Paediatric STEC-HUS and invasive pneumococcal infection (p-HUS)

Cobalamin deficiency (children), HSCT- or malignancy-associated TMA, malignant HT

(ii) Therapeutic plasma exchange (TPE)

First exclude paediatric STEC-HUS and p-HUS

Recommended in all other settings

Including TTP and aHUS (probably of no benefit in MCP-aHUS)

Controversial in adult STEC-HUS

Plasma infusion recommended in known congenital TTP

(iii) Eculizumab

aHUS

(iv) Steroids and/or rituximab

Possibly in acquired TTP and aHUS with factor H autoantibodies

(v) Renal transplantation for ESKD

STEC-HUS

MCP-aHUS

Living-related donation contraindicated

(vi) Prophylactic strategies in high-risk transplantation (i.e. non-MCP aHUS)

Intensive perioperative TPE

Eculizumab

Rituximab (for factor H autoantibodies)

Combined kidney–liver transplantation