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Langerhans' cell histiocytosis: Head and neck manifestations in children

✍ Scribed by Mr. Muhammad S. Quraishi; Mr. Alexander W. Blayney; Mr. David Walker; Mr. Fin B. Breatnach; Mr. Patrick J. Bradley


Publisher
John Wiley and Sons
Year
1995
Tongue
English
Weight
596 KB
Volume
17
Category
Article
ISSN
1043-3074

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✦ Synopsis


Background. Langerhans' cell histiocytosis (LCH) is an uncommon, poorly understood granulomatous disease, characterized by the idiopathic proliferation of Langerhan's cells or their marrow precursors. In 1985, the Philadelphia Workshop adopted the term "Langerhans' cell histiocytosis" (LCH) to differentiate it from reactive and neoplastic causes of histiocytosis.

Methods. This study includes 73 pediatric patients diagnosed with this condition in Dublin, Ireland, and Nottingham, England, during a 34-year period (1959 to 1993). These patients are reviewed with respect to clinical presentation, difficulty with making a histological diagnosis, their management, and outcome.

Resufrs. A total of 49 patients (67%) had head and neck involvement. Bony involvement was the most frequent sign, most frequently located in the skull. There were 11 deaths (15%) in this series, all associated with multisystem disease, and nine of these deaths were in children younger than 2 years of age.

Conclusions. The role of otolaryngologists is important in the early and accurate evaluation, staging, and diagnosis


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