Difference between revisions of "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''', and '''histiocytosis X'''. | '''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== | ==General== | ||
LCH is really three diseases - that happen to share the same histology:<ref>{{Ref PCPBoD8|338-9}}</ref> | |||
{| class="wikitable sortable" | |||
! 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== | ==Microscopic== | ||
Revision as of 12:38, 5 May 2012
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.
- Clusters of cells (histiocytes) with a reniform (kidney-shaped) nucleus and abundant foamy cytoplasm.
- +/-Eosinophils - often prominent.
Images:
- WC:
- www:
DDx:
- Kimura disease - eosinophilia.
- See lymph node pathology.
IHC
Electron microscopy
Etiology:
- Cell membrane invagination.[4]
Appearance:
- Electron dense, cytoplasmic tennis racket-like body.
Images:
See also
References
- ↑ 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.
- ↑ BN. 15 March 2011.
- ↑ Online 'Mendelian Inheritance in Man' (OMIM) 604862
- ↑ URL: http://path.upmc.edu/cases/case147/micro.html. Accessed on: 7 January 2012.
- ↑ URL: http://path.upmc.edu/cases/case298.html. Accessed on: 14 January 2012.