Korean J Pediatr Hematol Oncol.
2002 Oct;9(2):234-238.
Long-term Follow-up of an Evans Syndrome with Chronic, Refractory Course: Response to 5 Different Regimens
- Affiliations
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- 1Department of Pediatrics, Chonnam National University Medical School, Gwangju, Korea. hoonkook@chonnam.ac.kr
Abstract
- Evans syndrome is the combination of direct Coombs' positive hemolytic anemia and immune thrombocytopenic purpura, in the absence of a known underlying etiology. Being reported rarely in pediatric patients, the syndrome is characterized by periods of remission and exacerbation with viable responses to therapy. Management of the disease remains a challenge despite a variety of therapeutic trials. We experienced a 11-years old male patient of Evans syndrome who was initially presented as having an autoimmune hemolytic anemia 17 months before. Over the 5 years of follow-up, he had a chronic, relapsing courses, showing partial responses to a variety of therapeutic trials, including IVIG, oral prednisolone, methylprednisolone pulse therapy, cyclosporine A and vincristine. A brief review of the literature ensues with the case report.
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