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Difference between revisions of "Langerhans cell histiocytosis"
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==General== | ==General== | ||
LCH is really three diseases - that happen to share the same histology:<ref>{{Ref PCPBoD8|338-9}}</ref> | LCH is really four (or three) diseases (depending on how one classifies it) - that happen to share the same histology:<ref>{{Ref PCPBoD8|338-9}}</ref><ref name=pmid16295427>{{Cite journal | last1 = Chhabra | first1 = UD. | last2 = Desai | first2 = SS. | last3 = Jambhekar | first3 = NA. | title = Langerhans' cell histiocytosis: a clinicopathological study of 50 cases. | journal = Indian J Pathol Microbiol | volume = 47 | issue = 3 | pages = 370-6 | month = Jul | year = 2004 | doi = | PMID = 16295427 }}</ref> | ||
{| class="wikitable sortable" | {| class="wikitable sortable" | ||
! Disease | ! Disease | ||
| Line 19: | Line 19: | ||
|- | |- | ||
| Multifocal multisystem Langerhans cell histiocytosis | | Multifocal multisystem Langerhans cell histiocytosis | ||
| Letterer-Siwe disease | | multisystem LCH, Letterer-Siwe disease | ||
| outcome dependent on organ involved,<ref name=pmid21351807>{{Cite journal | last1 = Minkov | first1 = M. | title = Multisystem Langerhans cell histiocytosis in children: current treatment and future directions. | journal = Paediatr Drugs | volume = 13 | issue = 2 | pages = 75-86 | month = Apr | year = 2011 | doi = 10.2165/11538540-000000000-00000 | PMID = 21351807 }}</ref> natural history 2 year survival, 50% five year survival with treatment | | outcome dependent on organ involved,<ref name=pmid21351807>{{Cite journal | last1 = Minkov | first1 = M. | title = Multisystem Langerhans cell histiocytosis in children: current treatment and future directions. | journal = Paediatr Drugs | volume = 13 | issue = 2 | pages = 75-86 | month = Apr | year = 2011 | doi = 10.2165/11538540-000000000-00000 | PMID = 21351807 }}</ref> natural history 2 year survival, 50% five year survival with treatment | ||
| usu. children < 2 years old, rarely adults<ref name=pmid22470214>{{Cite journal | last1 = Garg | first1 = A. | last2 = Kumar | first2 = P. | title = Multisystem Langerhans cell histiocytosis in adult. | journal = Indian J Dermatol | volume = 57 | issue = 1 | pages = 58-60 | month = Jan | year = 2012 | doi = 10.4103/0019-5154.92683 | PMID = 22470214 }}</ref> | | usu. children < 2 years old, rarely adults<ref name=pmid22470214>{{Cite journal | last1 = Garg | first1 = A. | last2 = Kumar | first2 = P. | title = Multisystem Langerhans cell histiocytosis in adult. | journal = Indian J Dermatol | volume = 57 | issue = 1 | pages = 58-60 | month = Jan | year = 2012 | doi = 10.4103/0019-5154.92683 | PMID = 22470214 }}</ref> | ||
| multiple systems (skin, spleen, liver, lung, bone marrow) | | multiple systems (skin, spleen, liver, lung, bone marrow) | ||
| genetic | | possibly genetic ‡ | ||
|- | |- | ||
| Unifocal | | Unifocal Langerhans cell histiocytosis † | ||
| Eosinophilic granuloma | | Eosinophilic granuloma | ||
| may spontaneously regress, may cure with surgery | | may spontaneously regress, may cure with surgery | ||
| children (?) | | children (?) | ||
| usu. bone | | bone only | ||
| genetic | | possibly genetic ‡ | ||
|- | |||
| Multifocal unisystem Langerhans cell histiocytosis † | |||
| multifocal LCH, eosinophilic granuloma, Hand-Schuller-Christian syndrome = bone defect, diabetes insipidus & exopthalmos | |||
| may spontaneously regress, may cure with surgery (?) | |||
| children (?) | |||
| usu. bone; may be in: skin, lungs, stomach | |||
| possibly genetic ‡ | |||
|} | |} | ||
Note: | |||
* † Robbins lumps these groups together. | |||
* ‡ Incompletely understood. Somatic BRAF mutations identified in approximately half of the individuals.<ref name=pmid20519626>{{Cite journal | last1 = Badalian-Very | first1 = G. | last2 = Vergilio | first2 = JA. | last3 = Degar | first3 = BA. | last4 = MacConaill | first4 = LE. | last5 = Brandner | first5 = B. | last6 = Calicchio | first6 = ML. | last7 = Kuo | first7 = FC. | last8 = Ligon | first8 = AH. | last9 = Stevenson | first9 = KE. | title = Recurrent BRAF mutations in Langerhans cell histiocytosis. | journal = Blood | volume = 116 | issue = 11 | pages = 1919-23 | month = Sep | year = 2010 | doi = 10.1182/blood-2010-04-279083 | PMID = 20519626 }}</ref><ref name=pmid22017623>{{Cite journal | last1 = Badalian-Very | first1 = G. | last2 = Vergilio | first2 = JA. | last3 = Degar | first3 = BA. | last4 = Rodriguez-Galindo | first4 = C. | last5 = Rollins | first5 = BJ. | title = Recent advances in the understanding of Langerhans cell histiocytosis. | journal = Br J Haematol | volume = 156 | issue = 2 | pages = 163-72 | month = Jan | year = 2012 | doi = 10.1111/j.1365-2141.2011.08915.x | PMID = 22017623 }}</ref> | |||
==Microscopic== | ==Microscopic== | ||