Factor H is a control protein in the complements system. Autoantibodies against factor H can trigger an atypical hemolytic uremic syndrome (HUS). Total plasma exchange can be helpful by removing the autoantibodies.
Patient selection: atypical hemolytic uremic syndrome (HUS) with Factor H autoantibodies
The primary therapeutic approach is to administer eculizumab (humanized anti-C5) which blocks activation of the terminal complement cascade.
Total plasma exchange can be used if eculizumab is not available or if the patient cannot tolerate the therapy.
Target volume: 1.5 times total plasma volume
Replacement: fresh frozen plasma or albumin
Frequency: daily
Total plasma exchange is given in conjunction with rituximab or other immunosuppression, which reduces production of the autoantibody.
To read more or access our algorithms and calculators, please log in or register.
