Severe refractory idiopathic warm autoimmune haemolytic anaemia responsive to complement inhibition with eculizumab

BMJ Case Rep. 2018 Dec 13;11(1):e226429. doi: 10.1136/bcr-2018-226429.

Abstract

We report a case of severe idiopathic warm autoimmune haemolytic anaemia (wAIHA) which was initially poorly responsive to treatment with corticosteroids, intravenous immunoglobulin, mycophenolate mofetil and rituximab, and required transfusion of more than 30 units of red cells over 12 weeks. Off-label use of the terminal complement pathway inhibitor, eculizumab, led to rapid amelioration of the haemolysis, presumably by the inhibition of an intravascular component, and allowed time for slower acting immunosuppressive agent to take effect. This novel approach warrants further evaluation, given the poor prognosis of multirefractory wAIHA.

Keywords: drugs and medicines; haematology (drugs and medicines); haematology (incl blood transfusion); immunological products and vaccines; immunology.

Publication types

  • Case Reports

MeSH terms

  • Anemia, Hemolytic, Autoimmune / drug therapy*
  • Antibodies, Monoclonal, Humanized / administration & dosage*
  • Complement Membrane Attack Complex / antagonists & inhibitors
  • Female
  • Humans
  • Middle Aged
  • Off-Label Use*
  • Treatment Outcome

Substances

  • Antibodies, Monoclonal, Humanized
  • Complement Membrane Attack Complex
  • eculizumab