Skip Navigation LinksHome > July 2010 - Volume 32 - Issue 5 > Endolymphatic Non-Langerhans Cell Histiocytosis of the Laryn...
Journal of Pediatric Hematology/Oncology:
doi: 10.1097/MPH.0b013e3181d746eb
Online Articles: Original Articles

Endolymphatic Non-Langerhans Cell Histiocytosis of the Larynx: Report of an Uncommon Disease Manifestation

Classen, Carl Friedrich MD, PhD*; Haffner, Dieter MD, PhD*; Nizze, Horst MD, PhD; Möller, Peter MD, PhD; Prall, Friedrich MD, PhD

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Abstract

We report on an uncommon laryngeal non-Langerhans cell histiocytosis. An 11-year-old boy presented with a 6 months history of progressive breath inhibition. Magnetic resonance imaging showed diffuse laryngeal and local lymph node swelling. Histology first resembled sarcoidosis, however, corticosteroids were ineffective. Lymphoma, infection, immunodeficiency, and autoimmune disease were excluded. Six months later, biopsies were repeated, now showing numerous ectatic lymph vessels with clusters of histiocytes bearing stellate extensions and emperipolesis. S100 protein and CD1a were negative. Indomethacin treatment led to a gradual improvement. In conclusion, we observed a nonmalignant non-Langerhans cell endolymphatic reticulohistiocytosis, not fitting into any of the described categories.

© 2010 Lippincott Williams & Wilkins, Inc.

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