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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== | ||