Remission and recurrence free 2 years follow up in a patient with onset of factor H antibody associated atypical HUS under induction therapy with Plasmapheresis and introduction of maintenance therapy with i.v.IgG and MMF

Abstract

Objective: Factor H antibodies (FH Ab) develop in 6-10% of aHUS patients. The role of FH Ab in disease onset, progression and treatment is of critical interest for physicians and patients dealing with this unsolved problem. At present, evidence based therapy recommendations for this group of patients are missing. Many patients develop end stage renal disease (ESRD) and recurrence rates within the first year are up to 70%. Methods: A 15 year old girl was addmited to the hospital because of weakness, vommitinganddecreasing amount of urine. 6 days ago she had otitis. On examination slight oedema and normal blood pressure. Laboratory analysis showed anemia (Hb - 71 g/l), thrombocytopaenia (55x109/l), elevated serum creatinin concentration (1219 micromol/l), high LDH (2612 U/l), low haptoglobin (86 mg/l) and low C3 (0,38 g/l) levels. Hemodialysis (n=8) and plasmapheresis (regime as recommended in Guidlines of The European Paediatric Study Group for HUS) were started within 24 hours after addmition to the hospital. After 18 plasmapheresis sessions total remission (normalization of hematologic parameters and renal function) was obtained. 17 day later plasmapheresis sessions had to be started again because of hematologic relapse (Hb - 57 g/l, thrombocytes - 87x109/l, haptoglobin - 29 mg/l, C3 – 0.48 g/l, LDH - 418 U/l, serum creatinin concentration - 95 micromol/l) (total number of plasmapheresis - 36).At that time FHAb associated aHUSwas diagnosed and after remission maintanance therapy (3 months after the onset) with i.v.IgG 2g/kg body weight (days 0, 21, 41) and Mycophenolatemofetil (started on day 2; 600mg/m2 twice a day) was introduced. Repeated IgG infusion at 7 months after onset because of increasing FH Ab titers. Results: 24 months after onset of the disease the patient is on hematological and renal remission:Hb - 130 g/l, platelets - 281x109/l, LDH - 167 U/l, haptoglobin - 1283mg/l, serum creatinin - 66 micromol/[...].