Langerhans cell histiocytosis

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Langerhans cell histiocytosis, abbreviated LCH, is a rare genetic disorder of tissue macrophages. It broadly fits into the category of histiocytoses. It used to known as eosinophilic granuloma. It has been referred to by several eponyms - Hand-Schüller-Christian disease, Abt-Letterer-Siwe disease or Letterer-Siwe disease, and histiocytosis X.

General

LCH is really three diseases - that happen to share the same histology:[1]

Disease Other name(s) Prognosis Demographic Location Risks/cause
Pulmonary Langerhans cell histiocytosis Eosinophilic granuloma good with smoking cessation adults - smokers lung only; typically upper lung field due to smoking
Multifocal multisystem Langerhans cell histiocytosis Letterer-Siwe disease natural history 2 year survival, 50% five year survival with treatment children (?) multiple systems (skin, spleen, liver, lung, bone marrow) genetic
Unifocal and multifocal unisystem Langerhans cell histiocytosis Eosinophilic granuloma, Hand-Schuller-Christian syndrome = bone defect, diabetes insipidus & exopthalmos exicse, may spontaneously regress children (?) usu. bone, skin, lungs, stomach genetic (?)

Microscopic

Features:

  • Langerhans cells histiocytes - key feature.
    • Clusters of cells (histiocytes) with a reniform (kidney-shaped) nucleus and abundant foamy cytoplasm.
      • Nucleus may look like a "coffee bean", i.e. have nuclear grooves (similar to those in papillary thyroid carcinoma) -- appearance dependent on the rotation of the nucleus.[2]
      • Chromatin pattern: fine granular, light gray.
  • +/-Eosinophils - often prominent.

Images:

DDx:

IHC

  • CD1a +ve.
  • S100 +ve.
  • CD207 (AKA Langerin) +ve.[3]

Electron microscopy

Etiology:

  • Cell membrane invagination.[4]

Appearance:

  • Electron dense, cytoplasmic tennis racket-like body.

Images:

See also

References

  1. Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 338-9. ISBN 978-1416054542.
  2. BN. 15 March 2011.
  3. Online 'Mendelian Inheritance in Man' (OMIM) 604862
  4. URL: http://path.upmc.edu/cases/case147/micro.html. Accessed on: 7 January 2012.
  5. URL: http://path.upmc.edu/cases/case298.html. Accessed on: 14 January 2012.