Long-term Follow-up of an Evans Syndrome with Chronic, Refractory Course: Response to 5 Different Regimens.
- Author:
Chan Kyun OH
1
;
Hoon KOOK
;
Won Sang YOON
;
Chan Jong KIM
;
So Yeon KIM
;
Hyun Jeong KIM
;
Ho Song NAM
;
Tai Ju HWANG
Author Information
1. Department of Pediatrics, Chonnam National University Medical School, Gwangju, Korea. hoonkook@chonnam.ac.kr
- Publication Type:Case Report
- Keywords:
Evans syndrome;
IVIG;
Oral prednisolone;
Methylprednisolone;
Cyclosporin A;
Vincristine
- MeSH:
Anemia, Hemolytic;
Anemia, Hemolytic, Autoimmune;
Child;
Cyclosporine;
Follow-Up Studies*;
Humans;
Immunoglobulins, Intravenous;
Male;
Methylprednisolone;
Prednisolone;
Purpura, Thrombocytopenic, Idiopathic;
Vincristine
- From:Korean Journal of Pediatric Hematology-Oncology
2002;9(2):234-238
- CountryRepublic of Korea
- Language:Korean
-
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.
