Langerhans cell histiocytosis of bone.

M. A. Stull, M. J. Kransdorf, K. O. Devaney

Research output: Contribution to journalArticle

181 Citations (Scopus)

Abstract

Langerhans cell histiocytosis (LCH), previously called histiocytosis X, refers to a spectrum of disease characterized by idiopathic proliferation of histiocytes producing focal or systemic manifestations. Causes and pathogenesis remain unclear. However, recent studies suggest abnormal immune regulation as an important factor. The three classic syndromes may have considerable clinical overlap: eosinophilic granuloma, in which the disease is limited to bone in patients usually 5-15 years old; Hand-Schüller-Christian disease, characterized by multifocal bone lesions and extraskeletal involvement of the reticuloendothelial system (RES) usually seen in children 1-5 years old; and Letterer-Siwe disease, in which there is disseminated involvement of the RES with a fulminant clinical course in children less than 2 years old. Osseous involvement is typically in the flat bones, with lesions of the skull, pelvis, and ribs accounting for more than half of all lesions. About 30% of lesions are in long bones. Radiographic appearance of osseous LCH depends on site of involvement and phase of the disease. Early lesions appear aggressive with poorly defined margins and lamellated periosteal reaction. Late lesions appear well defined and may show sclerotic margins and expanded remodeled appearance.

Original languageEnglish (US)
Pages (from-to)801-823
Number of pages23
JournalRadiographics : a review publication of the Radiological Society of North America, Inc
Volume12
Issue number4
StatePublished - Jul 1992
Externally publishedYes

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Langerhans Cell Histiocytosis
Bone and Bones
Mononuclear Phagocyte System
Eosinophilic Granuloma
Histiocytes
Ribs
Pelvis
Skull
Hand

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Radiological and Ultrasound Technology

Cite this

Langerhans cell histiocytosis of bone. / Stull, M. A.; Kransdorf, M. J.; Devaney, K. O.

In: Radiographics : a review publication of the Radiological Society of North America, Inc, Vol. 12, No. 4, 07.1992, p. 801-823.

Research output: Contribution to journalArticle

Stull, M. A. ; Kransdorf, M. J. ; Devaney, K. O. / Langerhans cell histiocytosis of bone. In: Radiographics : a review publication of the Radiological Society of North America, Inc. 1992 ; Vol. 12, No. 4. pp. 801-823.
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