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Difference between revisions of "Langerhans cell histiocytosis"
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==General== | ==General== | ||
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> | ===Overview=== | ||
LCH is really four (or three) diseases (depending on how one classifies it) - that happen to share the same histology:<ref name=Ref_PCPBoD8_338-9>{{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 43: | Line 44: | ||
* † Robbins lumps these groups together. | * † 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> | * ‡ 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> | ||
===Clinical presentation=== | |||
Features - dependent on subtype:<ref name=Ref_PCPBoD8_338-9>{{Ref PCPBoD8|338-9}}</ref> | |||
*May present with fever, anemia, bone pain, bone fracture, diabetes insipidus, exophthalmos. | |||
*Can be an incidental finding. | |||
==Microscopic== | ==Microscopic== | ||