Management of Evans syndrome

Alice Norton, Irene Roberts
In: British Journal of Haematology · 2005 · vol. 132(2) , pp. 125–137 · doi:10.1111/j.1365-2141.2005.05809.x · PMID:16398647 · W2038137985
review OA: bronze CC0 ⤵ 2 in-corpus citations
🔓 Open OA copy View on OpenAlex View on PubMed View at publisher
⚙ AI-generated summary by gemini-2.5-flash-lite, 2026-08-03 ⓘ

Evans syndrome, a chronic autoimmune disorder of thrombocytopenia and hemolytic anemia, is typically managed with corticosteroids and IVIg, with relapses often treated by immunosuppressants, rituximab, splenectomy, or, in severe cases, stem cell transplantation.

One-sentence paraphrase of the abstract; not a substitute for reading it. No clinical advice. How this works

Abstract

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.

My notes (saved in your browser only)

Citation neighborhood (sparse)

Too few in-corpus citations on either side for a chart; here are the lists.

Cites (3)

References (75)

Source provenance

openalex
last seen: 2026-05-11T06:35:44.859312+00:00
License: CC0 · commercial use OK