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Langerhans cell histiocytosis

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Definition

noun

A rare type of histiocytosis involving the Langerhans cells that proliferate in excessive number and accumulate in various organs


Supplement

Histiocytosis refers to a disorder in which the number of histiocytes is atypically high. Histiocytes are reticular connective tissue macrophage or a dendritic cell. They came from the stem cells of the bone marrow, then, they migrate into the bloodstream as monocytes. When they reach and enter a certain organ they undergo differentiation to become histiocytes as influenced by growth factors, GM-CSF, TNF, and IL-4.1 A rare type of histiocytosis is the Langerhans cell histiocytosis. It refers to a group of disorders of histiocyte proliferation which includes letterer-siwe disease, hand-schueller-christian syndrome, and eosinophilic granuloma. Langerhans cells are components of the lesions. Its symptoms include inflammation, lethargy, fever, and weight loss. There is also a skin rash which varies, i.e. from scale erythematous to red papules. The patient may experience painful bone swelling, especially at the skull. The lytic bone lesion is a hallmark of Langerhans cell histiocytosis.2


Abbreviation / Acronym: LCH

Synonym(s):

  • Histiocytosis X
  • Histiocytosis X syndrome

See also:


Reference(s):
1 Histiocyte. Retrieved from [[1]].
2 Shreve, P. & Townsend, D. (2011). Clinical PET-CT in radiology integrated imaging in oncology. New York London: Springer. p.381.