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Evan's syndrome
Autoimmune hemolytic anemia & autoimmune thrombocytopenia.
Epidemiology: uncommon
Clinical manifestations:
- chronic & relapsing
Laboratory:
- Coomb's positive hemolytic anemia
- direct antiglobulin test (DAT, direct Coomb's test) is positive [3]
Management:
1) treatment is similar but less satisfactory than for patients with isolated autoimmune hemolytic anemia or autoimmune thrombocytopenia:
- glucocorticoids
- intravenous immune globulin
- rituximab induces remission in most patients, but sustained
- other immunosuppresive agents: cyclosporin, CellCept, vincristrine
- danazole
2) long term remissions after splenectomy less frequent than with ITP
3) for severe & refractory cases, stem cell transplantation may be an option for younger patients [2]
General
autoimmune disease
hemolytic anemia
thrombocytopenia
syndrome
References
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed)
Lippincott-Raven, Philadelphia, 1998, pg 414
- Norton A and Roberts I
Management of Evans syndrome
Br J Haematol 2006, 132:125
PMID: 16398647
- NEJM Knowledge+