Successful Treatment of an Adolescent Male with Severe Refractory Evans Syndrome Using Bortezomib-based Therapy

Tristan Knight, Yaddanapudi Ravindranath, Michael U. Callaghan

Research output: Contribution to journalArticlepeer-review

5 Scopus citations

Abstract

Evans syndrome is defined by bilineal autoimmune cytopenia, typically coombs positive hemolytic anemia and thrombocytopenia. Corticosteroids are the mainstay of treatment, with rituximab and/or mycophenolate mofetil often used in steroid-refractory cases. However, no treatment methodology has ever evaluated by a randomized clinical trial. We present a 15-year-old boy with Evans syndrome and common variable immunodeficiency who experienced a severe, refractory flare 16 months postsplenectomy. After failing to respond to multiple other agents, he achieved a durable response to a bortezomib-based regimen. Bortezomib may be a reasonable second or third line option, especially before high-morbidity therapies such as splenectomy or stem cell transplantation.

Original languageEnglish
Pages (from-to)E110-E113
JournalJournal of Pediatric Hematology/Oncology
Volume42
Issue number2
DOIs
StatePublished - Mar 1 2020
Externally publishedYes

Keywords

  • Evans syndrome
  • autoimmune cytopenia
  • bortezomib

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