Management of Evans syndrome.
Evans syndrome is an uncommon condition defined by the combination (either simultaneously or sequentially) of immune thrombocytopenia (ITP) and autoimmune haemolytic anaemia (AIHA) with a positive direct antiglobulin test (DAT) in the absence of known underlying aetiology. This condition generally runs a chronic course and is characterised by frequent exacerbations and remissions. First-line therapy is usually corticosteroids and/or intravenous immunoglobulin, to which most patients respond; however, relapse is frequent. Options for second-line therapy include immunosuppressive drugs, especially ciclosporin or mycophenolate mofetil; vincristine; danazol or a combination of these agents. More recently a small number of patients have been treated with rituximab, which induces remission in the majority although such responses are often sustained for <12 months and the long-term effects in children are unclear. Splenectomy may also be considered although long-term remissions are less frequent than in uncomplicated ITP. For very severe and refractory cases stem cell transplantation (SCT) offers the only chance of long-term cure. The limited data available suggest that allogeneic SCT may be superior to autologous SCT but both carry risks of severe morbidity and of transplant-related mortality. Cure following reduced-intensity conditioning has now been reported and should be considered for younger patients in the context of controlled clinical trials.
Norton
Alice
A
Paediatric Haematology, Department of Paediatrics, St Mary's Hospital, Paddington, London, UK.
Roberts
Irene
I
D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis / Q000628:therapy*; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000628:therapy*; D003937:Diagnosis, Differential; D006801:Humans; D011379:Prognosis; D013577:Syndrome; D013921:Thrombocytopenia / Q000175:diagnosis / Q000628:therapy*