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Infant Langerhans Cell Histiocytosis Presented as Isolated Splenomegaly

Other Title
비장비대와 불명열을 동반한 비장에 국한된 랑게르한스세포조직구증 1예
Authors
Shon, HJ | Jung, HJ  | Park, JE
Citation
Clinical pediatric hematology-oncology, 20(2). : 126-130, 2013
Journal Title
Clinical pediatric hematology-oncology
ISSN
2233-52502233-4580
Abstract
The isolated spleen involvement is a rare presentation of Langerhans cell histiocytosis (LCH) and can lead to a misdiagnosis. We report a case of LCH in an infant presentated isolated splenomegaly and prolonged fever. A 1-month-old girl admitted due to pe-techiae, presented with fever for a week and splenomegaly, and showed marginal im-provement on empirical treatment. The fever and progressive spleen enlargement per-sisted over 4 weeks and the cause remained unclear, even after thorough evaluation including bone marrow aspiration and biopsy. LCH was confirmed by splenectomy, and involvement of other organs was excluded. Splenectomy is valuable in the diagnosis of LCH presented with isolated splenomegaly without other organ involvement which may show more aggressive clinical course, but it should be limited to absolutely neces-sary indications.
Keywords
Appears in Collections:
Journal Papers > School of Medicine / Graduate School of Medicine > Pediatrics & Adolescent Medicine
Ajou Authors
박, 준은  |  정, 현주
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