Lymphoma & related disorders
Splenic hematogenous neoplasms
Langerhans cell histiocytosis

Topic Completed: 1 March 2013

Minor changes: 1 April 2020

Copyright: 2003-2020,, Inc.

PubMed Search: Langerhans cell histiocytosis[TI] spleen

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Cite this page: Mansouri J. Langerhans cell histiocytosis. website. Accessed August 3rd, 2020.
Definition / general
  • Normal counterpart is presumed to be mature Langerhans cell
  • Almost always due to systemic disease often involving spleen, liver, skin, bone and bone marrow; but may also present as a solitary lesion (e.g. eosinophilic granuloma) in bone or lung
  • Usually found only at autopsy in spleen, associated with fatal thrombocytopenia due to hypersplenism
  • Can occasionally be associated with T cell lymphoblastic leukemia
  • Langerhans cell sarcoma: cytologically malignant (Am J Dermatopathol 2006;28:36)
Gross description
  • Spleen shows multinodular involvement of red pulp
Microscopic (histologic) description
  • Usually involves red pulp
  • Large epithelioid-like cells with folded nuclei
Microscopic (histologic) images

Images hosted on other servers:

LCH - site unspecified

Positive stains
Electron microscopy description
  • Birbeck granules
Molecular / cytogenetics description
Molecular / cytogenetics images

Images hosted on other servers:

Immunofluorescence analysis of BRAF pathway activation in LCH

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