Evan's syndrome

Introduction

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

Management

• 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
• long term remissions after splenectomy less frequent than with ITP
• for severe & refractory cases, stem cell transplantation may be an option for younger patients

More general terms

• autoimmune disease
• hemolytic anemia
• thrombocytopenia
• syndrome

Patient information

Evan's syndrome patient information