Treatment of Atypical Hemolytic-Uremic Syndrome Without Anti-Factor H Antibodies

This protocol covers the management of atypical hemolytic-uremic syndrome (aHUS) in patients where anti-factor H antibodies have been excluded — a subgroup with distinct therapeutic considerations linked to complement pathway genetics.

Clinical Scenario

Atypical hemolytic-uremic syndrome without detectable anti-factor H antibodies. In Europe and North America, inherited defects of the alternative complement pathway are the chief underlying cause of aHUS in this presentation.

Treatment Approach

Management involves prompt initiation of plasma exchange as the primary intervention, preferred over plasma infusion as the initial plasma-based strategy.

The complete protocol — including the full treatment sequence, criteria for adjusting therapy, and response thresholds — is available via the link below.

Treatment Goals

The target is hematological remission: platelets > 100,000/μl, schistocytes < 2%, and LDH below the upper limit of normal — confirmed on two consecutive days.

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References

DOI: 10.1007/s00467-019-04233-7

Inherited defects of the alternative pathway are the chief cause of aHUS in Europe and North America.
Thus, PEX or plasma infusions remain the chief option for patients with aHUS, especially those with genetic defects in the complement pathway.
In the absence of eculizumab, we recommend prompt initiation of PEX in patients with aHUS.
For initial therapy, we recommend that PEX be preferred to plasma infusions.
We suggest that PEX be administered daily until hematological remission and then tapered over 4–6 weeks (Table 3).
Hematological remission: Platelets > 100,000/μl, schistocytes < 2%, LDH less than upper limit of normal on 2 consecutive days.

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