Systemic Epstein-Barr Virus-positive T-Cell Lymphoproliferative Disease of Childhood With Good Response to Steroid Therapy
- Affiliated Author(s)
- 김도훈
- Alternative Author(s)
- Kim, Do Hoon
- Journal Title
- Journal of Pediatric Hematology/Oncology
- ISSN
- 1077-4114
- Issued Date
- 2017
- Keyword
- EBV; T cells; lymphoproliferative disease; childhood; steroid
- Abstract
- Systemic Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disease of childhood is a rare disease and has a very fulminant clinical course with high mortality. A 21-month-old female patient was referred to our hospital with a 1 week history of fever and was subsequently diagnosed with systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood. After starting treatment with dexamethasone, she showed early defervescence and improvement of laboratory parameters, and has remained disease-free after stopping steroid treatment, although longer follow-up is necessary. Our report underscores the possibility that this disease entity may be heterogenous in terms of prognosis.
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