Today at Parkland Morning Report we discussed about the utility of the medication, eculizumab, for hemolytic uremic syndrome. Below are some interesting articles regarding treating thrombotic thrombocytopenic purpura, hemolytic uremic syndrome (HUS), and eculizumab. Key points below:
- Eculizumab is a recombinant, humanized, monoclonal anti-C5 antibody. It binds to C5 which halts the complement cascade and inhibits production of protein complexes that kill cells.
- Blocks terminal complement activation, thus patients may have higher risk to infections from encapsulated bacteria such as Neisseria meningitides, Haemophilus influenzae and pneumococci as vaccination is recommended.
- Used to treat paroysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS).
- Atypical HUS is distinguished from classic HUS in that it primarily involves the kidneys and seems to have a clear link to dysregulation of the complement system.
- In regards to administration, patients typically receive 4 weekly IV doses for induction, then a maintenance dose every 2 weeks for aHUS. Duration is unclear as some patient remain on therapy indefinitely. Alternative therapy is plasma therapy.
- Eculizumab side effects include constipation, nausea, vomiting, tachycardia, fever.
How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome
Update on hemolytic uremic syndrome: Diagnostic and therapeutic recommendations