Langerhans cell histiocytosis - Revision history

Michael: /* Images */

2016-04-25T03:32:12Z

Images

← Older revision		Revision as of 03:32, 25 April 2016
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	===Images===		===Images===
	<gallery>		<gallery>
	Image:~~Langerhans_cell_histiocytosis_~~-~~_very_high_mag~~.jpg \| LCH - ~~lymph node~~ - ~~very high~~ mag. (WC)		Image:Langerhans cell histiocytosis - very low mag.jpg\| LCH - very low mag. (WC/Nephron)
	Image:~~Langerhans_cell_histiocytosis_~~-~~_high_mag~~.jpg \| LCH - ~~lymph node~~ - high mag. (WC)		Image:Langerhans cell histiocytosis - low mag.jpg\| LCH - low mag. (WC/Nephron)
			Image:Langerhans cell histiocytosis - intermed mag.jpg\| LCH - intermed. mag. (WC/Nephron)
			Image:Langerhans cell histiocytosis - very high mag.jpg\| LCH - very high mag. (WC/Nephron)
	</gallery>		</gallery>
	www:		====www====
	*[http://path.upmc.edu/cases/case533.html LCH - several images (upmc.edu)].		*[http://path.upmc.edu/cases/case533.html LCH - several images (upmc.edu)].

Michael: /* Molecular */

2016-04-25T03:30:13Z

Molecular

← Older revision		Revision as of 03:30, 25 April 2016
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	==Molecular==		==Molecular==
	*Commonly have BRAF mutations ~ 40-70% of cases.<ref name=pmid26980021>{{Cite journal \| last1 = Alayed \| first1 = K. \| last2 = Medeiros \| first2 = LJ. \| last3 = Patel \| first3 = KP. \| last4 = Zuo \| first4 = Z. \| last5 = Li \| first5 = S. \| last6 = Verma \| first6 = S. \| last7 = Galbincea \| first7 = J. \| last8 = Cason \| first8 = RC. \| last9 = Luthra \| first9 = R. \| title = BRAF and MAP2K1 mutations in Langerhans cell histiocytosis: a study of 50 cases. \| journal = Hum Pathol \| volume = \| issue = \| pages = \| month = Feb \| year = 2016 \| doi = 10.1016/j.humpath.2015.12.029 \| PMID = 26980021 }}</ref>		*Commonly have BRAF mutations ~ 40-70% of cases.<ref name=pmid26980021>{{Cite journal \| last1 = Alayed \| first1 = K. \| last2 = Medeiros \| first2 = LJ. \| last3 = Patel \| first3 = KP. \| last4 = Zuo \| first4 = Z. \| last5 = Li \| first5 = S. \| last6 = Verma \| first6 = S. \| last7 = Galbincea \| first7 = J. \| last8 = Cason \| first8 = RC. \| last9 = Luthra \| first9 = R. \| title = BRAF and MAP2K1 mutations in Langerhans cell histiocytosis: a study of 50 cases. \| journal = Hum Pathol \| volume = \| issue = \| pages = \| month = Feb \| year = 2016 \| doi = 10.1016/j.humpath.2015.12.029 \| PMID = 26980021 }}</ref>
	**''[[BRAF V600E mutation\|V600E ~~mutations~~]]'' ~~are~~ the most common.<ref name=pmid27094161>{{Cite journal \| last1 = Tatsuno \| first1 = M. \| last2 = Shioda \| first2 = Y. \| last3 = Iwafuchi \| first3 = H. \| last4 = Yamazaki \| first4 = S. \| last5 = Iijima \| first5 = K. \| last6 = Takahashi \| first6 = C. \| last7 = Ono \| first7 = H. \| last8 = Uchida \| first8 = K. \| last9 = Okamura \| first9 = O. \| title = BRAF V600 mutations in Langerhans cell histiocytosis with a simple and unique assay. \| journal = Diagn Pathol \| volume = 11 \| issue = 1 \| pages = 39 \| month = \| year = 2016 \| doi = 10.1186/s13000-016-0489-z \| PMID = 27094161 }}</ref>		**The ''[[BRAF V600E mutation\|V600E mutation]]'' is the most common BRAF mutation.<ref name=pmid27094161>{{Cite journal \| last1 = Tatsuno \| first1 = M. \| last2 = Shioda \| first2 = Y. \| last3 = Iwafuchi \| first3 = H. \| last4 = Yamazaki \| first4 = S. \| last5 = Iijima \| first5 = K. \| last6 = Takahashi \| first6 = C. \| last7 = Ono \| first7 = H. \| last8 = Uchida \| first8 = K. \| last9 = Okamura \| first9 = O. \| title = BRAF V600 mutations in Langerhans cell histiocytosis with a simple and unique assay. \| journal = Diagn Pathol \| volume = 11 \| issue = 1 \| pages = 39 \| month = \| year = 2016 \| doi = 10.1186/s13000-016-0489-z \| PMID = 27094161 }}</ref>
	**MAP2K1 mutations are often found in the cases without BRAF mutations.<ref name=pmid26980021/><ref>{{Cite journal \| last1 = Chakraborty \| first1 = R. \| last2 = Hampton \| first2 = OA. \| last3 = Shen \| first3 = X. \| last4 = Simko \| first4 = SJ. \| last5 = Shih \| first5 = A. \| last6 = Abhyankar \| first6 = H. \| last7 = Lim \| first7 = KP. \| last8 = Covington \| first8 = KR. \| last9 = Trevino \| first9 = L. \| title = Mutually exclusive recurrent somatic mutations in MAP2K1 and BRAF support a central role for ERK activation in LCH pathogenesis. \| journal = Blood \| volume = 124 \| issue = 19 \| pages = 3007-15 \| month = Nov \| year = 2014 \| doi = 10.1182/blood-2014-05-577825 \| PMID = 25202140 }}</ref>		**MAP2K1 mutations are often found in the cases without BRAF mutations.<ref name=pmid26980021/><ref>{{Cite journal \| last1 = Chakraborty \| first1 = R. \| last2 = Hampton \| first2 = OA. \| last3 = Shen \| first3 = X. \| last4 = Simko \| first4 = SJ. \| last5 = Shih \| first5 = A. \| last6 = Abhyankar \| first6 = H. \| last7 = Lim \| first7 = KP. \| last8 = Covington \| first8 = KR. \| last9 = Trevino \| first9 = L. \| title = Mutually exclusive recurrent somatic mutations in MAP2K1 and BRAF support a central role for ERK activation in LCH pathogenesis. \| journal = Blood \| volume = 124 \| issue = 19 \| pages = 3007-15 \| month = Nov \| year = 2014 \| doi = 10.1182/blood-2014-05-577825 \| PMID = 25202140 }}</ref>

Michael: /* Molecular */

2016-04-25T03:28:15Z

Molecular

← Older revision		Revision as of 03:28, 25 April 2016
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	==Molecular==		==Molecular==
	*Commonly have [[BRAF mutations]] ~ 40-70% of cases.<ref name=pmid26980021>{{Cite journal \| last1 = Alayed \| first1 = K. \| last2 = Medeiros \| first2 = LJ. \| last3 = Patel \| first3 = KP. \| last4 = Zuo \| first4 = Z. \| last5 = Li \| first5 = S. \| last6 = Verma \| first6 = S. \| last7 = Galbincea \| first7 = J. \| last8 = Cason \| first8 = RC. \| last9 = Luthra \| first9 = R. \| title = BRAF and MAP2K1 mutations in Langerhans cell histiocytosis: a study of 50 cases. \| journal = Hum Pathol \| volume = \| issue = \| pages = \| month = Feb \| year = 2016 \| doi = 10.1016/j.humpath.2015.12.029 \| PMID = 26980021 }}</ref>		*Commonly have BRAF mutations ~ 40-70% of cases.<ref name=pmid26980021>{{Cite journal \| last1 = Alayed \| first1 = K. \| last2 = Medeiros \| first2 = LJ. \| last3 = Patel \| first3 = KP. \| last4 = Zuo \| first4 = Z. \| last5 = Li \| first5 = S. \| last6 = Verma \| first6 = S. \| last7 = Galbincea \| first7 = J. \| last8 = Cason \| first8 = RC. \| last9 = Luthra \| first9 = R. \| title = BRAF and MAP2K1 mutations in Langerhans cell histiocytosis: a study of 50 cases. \| journal = Hum Pathol \| volume = \| issue = \| pages = \| month = Feb \| year = 2016 \| doi = 10.1016/j.humpath.2015.12.029 \| PMID = 26980021 }}</ref>
	**''~~V600~~ mutations'' are the most common.<ref name=pmid27094161>{{Cite journal \| last1 = Tatsuno \| first1 = M. \| last2 = Shioda \| first2 = Y. \| last3 = Iwafuchi \| first3 = H. \| last4 = Yamazaki \| first4 = S. \| last5 = Iijima \| first5 = K. \| last6 = Takahashi \| first6 = C. \| last7 = Ono \| first7 = H. \| last8 = Uchida \| first8 = K. \| last9 = Okamura \| first9 = O. \| title = BRAF V600 mutations in Langerhans cell histiocytosis with a simple and unique assay. \| journal = Diagn Pathol \| volume = 11 \| issue = 1 \| pages = 39 \| month = \| year = 2016 \| doi = 10.1186/s13000-016-0489-z \| PMID = 27094161 }}</ref>		**''[[BRAF V600E mutation\|V600E mutations]]'' are the most common.<ref name=pmid27094161>{{Cite journal \| last1 = Tatsuno \| first1 = M. \| last2 = Shioda \| first2 = Y. \| last3 = Iwafuchi \| first3 = H. \| last4 = Yamazaki \| first4 = S. \| last5 = Iijima \| first5 = K. \| last6 = Takahashi \| first6 = C. \| last7 = Ono \| first7 = H. \| last8 = Uchida \| first8 = K. \| last9 = Okamura \| first9 = O. \| title = BRAF V600 mutations in Langerhans cell histiocytosis with a simple and unique assay. \| journal = Diagn Pathol \| volume = 11 \| issue = 1 \| pages = 39 \| month = \| year = 2016 \| doi = 10.1186/s13000-016-0489-z \| PMID = 27094161 }}</ref>
	**MAP2K1 mutations are often found in the cases without BRAF mutations.<ref name=pmid26980021/><ref>{{Cite journal \| last1 = Chakraborty \| first1 = R. \| last2 = Hampton \| first2 = OA. \| last3 = Shen \| first3 = X. \| last4 = Simko \| first4 = SJ. \| last5 = Shih \| first5 = A. \| last6 = Abhyankar \| first6 = H. \| last7 = Lim \| first7 = KP. \| last8 = Covington \| first8 = KR. \| last9 = Trevino \| first9 = L. \| title = Mutually exclusive recurrent somatic mutations in MAP2K1 and BRAF support a central role for ERK activation in LCH pathogenesis. \| journal = Blood \| volume = 124 \| issue = 19 \| pages = 3007-15 \| month = Nov \| year = 2014 \| doi = 10.1182/blood-2014-05-577825 \| PMID = 25202140 }}</ref>		**MAP2K1 mutations are often found in the cases without BRAF mutations.<ref name=pmid26980021/><ref>{{Cite journal \| last1 = Chakraborty \| first1 = R. \| last2 = Hampton \| first2 = OA. \| last3 = Shen \| first3 = X. \| last4 = Simko \| first4 = SJ. \| last5 = Shih \| first5 = A. \| last6 = Abhyankar \| first6 = H. \| last7 = Lim \| first7 = KP. \| last8 = Covington \| first8 = KR. \| last9 = Trevino \| first9 = L. \| title = Mutually exclusive recurrent somatic mutations in MAP2K1 and BRAF support a central role for ERK activation in LCH pathogenesis. \| journal = Blood \| volume = 124 \| issue = 19 \| pages = 3007-15 \| month = Nov \| year = 2014 \| doi = 10.1182/blood-2014-05-577825 \| PMID = 25202140 }}</ref>

Michael at 03:27, 25 April 2016

2016-04-25T03:27:15Z

← Older revision		Revision as of 03:27, 25 April 2016
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	*S100 +ve.		*S100 +ve.
	*CD207 ([[AKA]] Langerin) +ve.<ref name=omim604862>{{OMIM\|604862}}</ref>		*CD207 ([[AKA]] Langerin) +ve.<ref name=omim604862>{{OMIM\|604862}}</ref>

			==Molecular==
			*Commonly have [[BRAF mutations]] ~ 40-70% of cases.<ref name=pmid26980021>{{Cite journal \| last1 = Alayed \| first1 = K. \| last2 = Medeiros \| first2 = LJ. \| last3 = Patel \| first3 = KP. \| last4 = Zuo \| first4 = Z. \| last5 = Li \| first5 = S. \| last6 = Verma \| first6 = S. \| last7 = Galbincea \| first7 = J. \| last8 = Cason \| first8 = RC. \| last9 = Luthra \| first9 = R. \| title = BRAF and MAP2K1 mutations in Langerhans cell histiocytosis: a study of 50 cases. \| journal = Hum Pathol \| volume = \| issue = \| pages = \| month = Feb \| year = 2016 \| doi = 10.1016/j.humpath.2015.12.029 \| PMID = 26980021 }}</ref>
			**''V600 mutations'' are the most common.<ref name=pmid27094161>{{Cite journal \| last1 = Tatsuno \| first1 = M. \| last2 = Shioda \| first2 = Y. \| last3 = Iwafuchi \| first3 = H. \| last4 = Yamazaki \| first4 = S. \| last5 = Iijima \| first5 = K. \| last6 = Takahashi \| first6 = C. \| last7 = Ono \| first7 = H. \| last8 = Uchida \| first8 = K. \| last9 = Okamura \| first9 = O. \| title = BRAF V600 mutations in Langerhans cell histiocytosis with a simple and unique assay. \| journal = Diagn Pathol \| volume = 11 \| issue = 1 \| pages = 39 \| month = \| year = 2016 \| doi = 10.1186/s13000-016-0489-z \| PMID = 27094161 }}</ref>
			**MAP2K1 mutations are often found in the cases without BRAF mutations.<ref name=pmid26980021/><ref>{{Cite journal \| last1 = Chakraborty \| first1 = R. \| last2 = Hampton \| first2 = OA. \| last3 = Shen \| first3 = X. \| last4 = Simko \| first4 = SJ. \| last5 = Shih \| first5 = A. \| last6 = Abhyankar \| first6 = H. \| last7 = Lim \| first7 = KP. \| last8 = Covington \| first8 = KR. \| last9 = Trevino \| first9 = L. \| title = Mutually exclusive recurrent somatic mutations in MAP2K1 and BRAF support a central role for ERK activation in LCH pathogenesis. \| journal = Blood \| volume = 124 \| issue = 19 \| pages = 3007-15 \| month = Nov \| year = 2014 \| doi = 10.1182/blood-2014-05-577825 \| PMID = 25202140 }}</ref>

	==Electron microscopy==		==Electron microscopy==

Michael at 21:47, 15 April 2016

2016-04-15T21:47:02Z

← Older revision		Revision as of 21:47, 15 April 2016
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			[[Image:Langerhans cell histiocytosis - very high mag.jpg\|thumb\|right\|Langerhans cell histiocytosis. [[H&E stain]]. (WC/Nephron)]]
	'''Langerhans cell histiocytosis''', abbreviated '''LCH''', is a rare disorder of tissue macrophages. It broadly fits into the category of ''[[histiocytoses]]''. It used to known as ''eosinophilic granuloma''.		'''Langerhans cell histiocytosis''', abbreviated '''LCH''', is a rare disorder of tissue macrophages. It broadly fits into the category of ''[[histiocytoses]]''. It used to known as ''eosinophilic granuloma''.

Michael at 06:25, 23 December 2015

2015-12-23T06:25:21Z

← Older revision		Revision as of 06:25, 23 December 2015
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	It has been referred to by several eponyms - '''Hand-Schüller-Christian disease''', '''Abt-Letterer-Siwe disease''' or '''Letterer-Siwe disease''', and '''histiocytosis X'''.		It has been referred to by several eponyms - '''Hand-Schüller-Christian disease''', '''Abt-Letterer-Siwe disease''' or '''Letterer-Siwe disease''', and '''histiocytosis X'''.

			This article deals with LCH in general. A separate article exists for ''[[pulmonary Langerhans cell histiocytosis]]''.

	==General==		==General==

Michael: /* Microscopic */

2013-06-04T01:51:08Z

Microscopic

← Older revision		Revision as of 01:51, 4 June 2013
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	*+/-Other inflammatory cells - neutrophils, plasma cells (uncommon).		*+/-Other inflammatory cells - neutrophils, plasma cells (uncommon).
	*+/-Multinucleated giant cells - uncommon.		*+/-Multinucleated giant cells - uncommon.

	~~Images:~~
	*[[WC]]:
	**[http://commons.wikimedia.org/wiki/File:Langerhans_cell_histiocytosis_-_very_high_mag.jpg LCH - lymph node - very high mag. (WC)].
	**[http://commons.wikimedia.org/wiki/File:Langerhans_cell_histiocytosis_-_high_mag.jpg LCH - lymph node - high mag. (WC)].
	*www:
	**[http://path.upmc.edu/cases/case533.html LCH - several images (upmc.edu)].

	DDx:		DDx:
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	*See ''[[lymph node pathology]]''.		*See ''[[lymph node pathology]]''.
	*See ''[[Long_power_list#Many_eosinophils\|lesions with many eosinophils]]''.		*See ''[[Long_power_list#Many_eosinophils\|lesions with many eosinophils]]''.

			===Images===
			<gallery>
			Image:Langerhans_cell_histiocytosis_-_very_high_mag.jpg \| LCH - lymph node - very high mag. (WC)
			Image:Langerhans_cell_histiocytosis_-_high_mag.jpg \| LCH - lymph node - high mag. (WC)
			</gallery>
			www:
			*[http://path.upmc.edu/cases/case533.html LCH - several images (upmc.edu)].

	==IHC==		==IHC==

Michael: /* Microscopic */

2012-05-05T13:23:25Z

Microscopic

← Older revision		Revision as of 13:23, 5 May 2012
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	==Microscopic==		==Microscopic==
	Features:		Features:<ref name=pmid16295427/>
	*Langerhans cells histiocytes - '''key feature'''.		*Langerhans cells histiocytes - '''key feature'''.
	**Clusters of cells (histiocytes) with a reniform (kidney-shaped) nucleus and abundant foamy cytoplasm.		**Clusters of cells (histiocytes) with a reniform (kidney-shaped) nucleus and abundant foamy cytoplasm.
	***Nucleus may look like a "[[coffee bean nucleus\|coffee bean]]", i.e. have nuclear grooves (similar to those in [[papillary thyroid carcinoma]]) -- appearance dependent on the rotation of the nucleus.<ref>BN. 15 March 2011.</ref>		***Nucleus may look like a "[[coffee bean nucleus\|coffee bean]]", i.e. have nuclear grooves (similar to those in [[papillary thyroid carcinoma]]) -- appearance dependent on the rotation of the nucleus.<ref>BN. 15 March 2011.</ref> May be called "buttock cells".
	***Chromatin pattern: fine granular, light gray.		***Chromatin pattern: fine granular, light gray.
	*+/-Eosinophils - often prominent.		*+/-Eosinophils - often prominent.
			*+/-Fibrosis - common.
			*+/-Other inflammatory cells - neutrophils, plasma cells (uncommon).
			*+/-Multinucleated giant cells - uncommon.

	Images:		Images:

Michael at 13:16, 5 May 2012

2012-05-05T13:16:47Z

← Older revision		Revision as of 13:16, 5 May 2012
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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''' or '''Letterer-Siwe disease''', and '''histiocytosis X'''.		'''Langerhans cell histiocytosis''', abbreviated '''LCH''', is a rare 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==

Michael: /* General */

2012-05-05T13:16:00Z

General

← Older revision		Revision as of 13:16, 5 May 2012
Line 2:		Line 2:

	==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
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	* † 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==