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Long-term Follow-up of an Evans Syndrome with Chronic, Refractory Course: Response to 5 Different Regimens / 대한소아혈액종양학회지
Korean Journal of Pediatric Hematology-Oncology ; : 234-238, 2002.
Article in Korean | WPRIM | ID: wpr-59292
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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Full text: Available Index: WPRIM (Western Pacific) Main subject: Vincristine / Methylprednisolone / Prednisolone / Follow-Up Studies / Purpura, Thrombocytopenic, Idiopathic / Immunoglobulins, Intravenous / Cyclosporine / Anemia, Hemolytic / Anemia, Hemolytic, Autoimmune Type of study: Observational study / Prognostic study Limits: Child / Humans / Male Language: Korean Journal: Korean Journal of Pediatric Hematology-Oncology Year: 2002 Type: Article

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Full text: Available Index: WPRIM (Western Pacific) Main subject: Vincristine / Methylprednisolone / Prednisolone / Follow-Up Studies / Purpura, Thrombocytopenic, Idiopathic / Immunoglobulins, Intravenous / Cyclosporine / Anemia, Hemolytic / Anemia, Hemolytic, Autoimmune Type of study: Observational study / Prognostic study Limits: Child / Humans / Male Language: Korean Journal: Korean Journal of Pediatric Hematology-Oncology Year: 2002 Type: Article