علاج متلازمة انحلال الدم اليوريمية اللانمطية بدون أجسام مضادة لعامل H

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.