Lymph nodes - not lymphoma
Neoplasms (not lymphoma)
Langerhans cell histiocytosis

Topic Completed: 1 June 2006

Revised: 4 November 2019

Copyright: (c) 2003-2018,, Inc.

PubMed Search: Langerhans cell histiocytosis[TI] lymph node full text[sb]

See also: Bone and Lung tumor chapters

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Cite this page: DePond W. Langerhans cell histiocytosis. website. Accessed November 21st, 2019.
Definition / general
  • Also called eosinophilic granuloma, histiocytosis X, Langerhans cell granulomatosis
  • Rare disorder of Langerhans cells
  • Variable clinical picture with single or multiple lesions or disseminated disease
  • Nodal involvement may be sole manifestation of disease or associated with systemic disease
  • May also have nodal involvement by lymphoma
  • Rarely have frankly malignant cells (Langerhans cell sarcoma) associated with more aggressive clinical behavior
  • Hand-Schüller-Christian disease: term used previously for more indolent disease of children and young adults
  • Hashitmoto-Pritzker disease: congenital, self limited form
  • Letterer-Siwe disease: systemic disease in infants
  • Langerhans cells capture antigens and present them to lymphocytes; are considered an immune system accessory cell
  • Langerhans cells are normally present as a few cells in thymus, lymph nodes and skin
Case reports
Microscopic (histologic) description
  • Partial effacement of lymph node with preservation of follicular centers but distension of nodal sinuses by Langerhans cells, which are 12 - 15 microns in diameter with abundant, pale eosinophilic cytoplasm, irregular and elongated nuclei with prominent nuclear grooves and folds, fine chromatin and indistinct nucleoli
  • Occasionally multinucleated
  • Sinuses commonly have foci of necrosis, often surrounded by rim of eosinophils
  • Rarely eosinophilic abscess incite a granulomatous response (J Clin Pathol 2006;59:548)
  • Variable mitotic activity
Microscopic (histologic) images

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AFIP images:

With Hodgkin lymphoma

Involving spleen



Nuclear and cytoplasmic staining S100

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Lymph node

Cytology description
Positive stains
Negative stains
Electron microscopy description
  • Irregular cell membrane and nucleus, Birbeck granules (elongated, zipperlike cytoplasmic structure, 200 - 400 x 33 nm with double outer sheath), variable lysosomes, no cell junctions
Electron microscopy images

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Birbeck granule

Molecular / cytogenetics description
  • Variable clonality
Additional references
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