Open AccessSevere refractory idiopathic warm autoimmune haemolytic anaemia responsive to complement inhibition with eculizumab
Author(s) -
Lucy Neave,
Andrew J. Wilson,
Maxine Lissack,
Marie Scully
Publication year - 2018
Publication title -
bmj case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.231
H-Index - 26
ISSN - 1757-790X
DOI - 10.1136/bcr-2018-226429
Subject(s) - eculizumab , medicine , rituximab , haemolysis , refractory (planetary science) , immunology , autoimmune hemolytic anemia , hemolysis , complement component 5 , antibody , complement system , physics , astrobiology
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.