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KMID : 0387820020090020234
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Clinical Pediatric Hematology-Oncology
2002 Volume.9 No. 2 p.234 ~ p.238
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Long-term Follow-up of an Evans Syndrome with Chronic, Refractory Course: Response to 5 Different Regimens
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Oh Chan-kyun
Kook Hoon
Yoon Won-Sang
Kim Chan-Jong
Kim So-Youn
Kim Hyun-Jung
Nam Ho-Song
Hwang Tai-Ju
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Abstract
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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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KEYWORD
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Evans Syndrome, IVIG, Oral prednisolone, Methylprednisolone, Cy-closporin A, Vincristine
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