علاج متلازمة انحلال الدم اليوريمية بأجسام مضادة لعامل H لدى الأطفال الذين تتراوح أعمارهم بين 5 و15 عامًا

References

DOI: 10.1007/s00467-019-04233-7

• Anti-FH antibody-associated illness constitutes ~ 50% of pediatric patients with aHUS in India, chiefly affecting children between the age of 5–15 years.
• We recommend a combination of prompt PEX (with fresh frozen plasma as replacement fluid) and immunosuppressive therapy for patients with anti-FH antibodies.
• We suggest daily PEX until hematological remission and then taper over 3–5 weeks.
• Since antibodies are chiefly IgG, 5–7 PEX achieve 80% reduction in antibody titers.
• Hematological remission: Platelets > 100,000/μl, schistocytes < 2%, LDH less than upper limit of normal on 2 consecutive days.