Practica oto-rhino-laryngologica. Suppl.
Online ISSN : 2185-1557
Print ISSN : 0912-1870
ISSN-L : 0912-1870
Festschrift for Professor Katsuhisa Ikeda In Honor 5 Years of Charmanship, Department of Otolaryngology, Juntendo University School of Medicine
A Case Report of Multi-System Multi-Site Type Langerhans Cell Histiocytosis
Misato KasaiEiko NakazawaYoko AzusawaSin ItoGen SugitaKyoko TazakiTakeshi KusunokiHiroshi YoshikawaKatsuhisa Ikeda
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2010 Volume 126 Pages 63-67

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Abstract

Langerhans cell histiocytosis (LCH) has been known to show a number of proliferating histiocytic cells that were similar in feature to Lanngerhans cells. LCH has been classified into three types, namely Single-System Single-Site (SS), Single-System Multi-Site (SM), and Multi-System Multi-Site (MM). A 9-month-old male infant with LCH (MM type) was treated in our hospital. Eruptions, the hard palate tumor and otorrhea were observed at the first examination. The CT scan showed destruction of the hard palate, maxillary sinus and temporal bone. We confirmed the diagnosis of LCH with a skin biopsy. The immunohistolgical findings showed a positive reaction for S-100 protein. Moreover, electromicoscopic observation identified Birbeck granules as one of the main characteristics of LCH. Our patient underwent chemotherapy according to the prtocol of the Japan LCH Study Group (JLSG, 1996). This therapy was able to treat the lesions of both bones and skin at 7 months after the JLSG chemotherapy of regimen.

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© 2010 The Society of Practical Otolaryngology
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