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Spleen (view source)

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[[Image:Gray1188.png|thumb|300px|Spleen.]]
The '''spleen''' is a forgotten organ.
The '''spleen''' is a forgotten organ.


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*Red pulp - [[red blood cells]].
*Red pulp - [[red blood cells]].
*White pulp - white blood cells.
*White pulp - white blood cells.
*Marginal zone - between red pulp and white pulp.<ref>{{Ref WMSP|589}}</ref>
===Images===
<gallery>
Image:Splenic_hamartoma_-_very_low_mag.jpg | Normal spleen (left) and splenic hamartoma (right). H&E stain. (WC)
</gallery>
==Sign out==
===Micro===
Sections show unremarkable splenic parenchyma. The nodules of white pulp are well-spaced and have germinal center formation.  The red pulp has a normal vascularity and does not appear to be expanded.  No nuclear atypia is apparent.
=Gross pathology=
The [[grossing]] of the spleen is dealt with in [[splenectomy grossing]].
===Overview of classic gross findings===
{| class = "wikitable sortable"
! Terminology
! Etiology
! Description
! Other
|-
|Ruptured spleen
| trauma
| hemorrhagic, capsule disrupted
| possible assocations [[mononucleosis]], medical procedure - esp. colonoscopy,<ref name=pmid22889306>{{Cite journal  | last1 = Aubrey-Bassler | first1 = FK. | last2 = Sowers | first2 = N. | title = 613 cases of splenic rupture without risk factors or previously diagnosed disease: a systematic review. | journal = BMC Emerg Med | volume = 12 | issue = 1 | pages = 11 | month = Aug | year = 2012 | doi = 10.1186/1471-227X-12-11 | PMID = 22889306 }}</ref> others
|-
|"Lardaceous spleen"<ref name=Ref_Klatt91>{{Ref Klatt|91}}</ref>
| [[amyloidosis]]
| waxy, pale, grey
| also see ''sago spleen''
|-
|"Sago spleen"
| [[amyloidosis]]
| nodular, tapioca-like appearance
| also see lardaceous spleen
|-
|[[Splenic infarct]]
| vascular occulsion
| wedge shaped -- periphery
| -
|-
|}
Images:
*[http://www.pathguy.com/lectures/sagolard.gif Amyloidosis of the spleen - schematic (pathguy.com)].
*[http://commons.wikimedia.org/wiki/File:Tapioca_pudding-3.jpg Tapioca pudding (WC)].


=Splenic enlargement=
=Splenic enlargement=
*Portal hypertension (often due to [[cirrhosis]]).
:''Splenomegaly'' redirects here.''
*Lymphoma:
*[[Portal hypertension]] (often due to [[cirrhosis]]).
**[[Hairy cell leukemia]].
*Lymphoma - see below.
**[[Splenic marginal zone lymphoma]].<ref name=pmid20350661>{{Cite journal  | last1 = Bennett | first1 = M. | last2 = Schechter | first2 = GP. | title = Treatment of splenic marginal zone lymphoma: splenectomy versus rituximab. | journal = Semin Hematol | volume = 47 | issue = 2 | pages = 143-7 | month = Apr | year = 2010 | doi = 10.1053/j.seminhematol.2010.01.004 | PMID = 20350661 }}</ref>
**Splenic diffuse red pulp small B cell lymphoma.<ref name=pmid20677173>{{cite journal |author=Baseggio L, Traverse-Glehen A, Callet-Bauchu E, ''et al.'' |title=Relevance of a scoring system including CD11c expression in the identification of splenic diffuse red pulp small B-cell lymphoma (SRPL) |journal=Hematol Oncol |volume=29 |issue=1 |pages=47–51 |year=2011 |month=March |pmid=20677173 |doi=10.1002/hon.957 |url=}}</ref>
*Mononucleosis ([[EBV]] infection).
*Mononucleosis ([[EBV]] infection).
*[[Hemophagocytic syndrome]].<ref name=pmid20127750>{{Cite journal  | last1 = Maakaroun | first1 = NR. | last2 = Moanna | first2 = A. | last3 = Jacob | first3 = JT. | last4 = Albrecht | first4 = H. | title = Viral infections associated with haemophagocytic syndrome. | journal = Rev Med Virol | volume = 20 | issue = 2 | pages = 93-105 | month = Mar | year = 2010 | doi = 10.1002/rmv.638 | PMID = 20127750 }}
*[[Hemophagocytic syndrome]].<ref name=pmid20127750>{{Cite journal  | last1 = Maakaroun | first1 = NR. | last2 = Moanna | first2 = A. | last3 = Jacob | first3 = JT. | last4 = Albrecht | first4 = H. | title = Viral infections associated with haemophagocytic syndrome. | journal = Rev Med Virol | volume = 20 | issue = 2 | pages = 93-105 | month = Mar | year = 2010 | doi = 10.1002/rmv.638 | PMID = 20127750 }}
</ref>
</ref>
==Lymphoid neoplasms of the spleen==
Lymphomas of the spleen in order of prevalence - in a series of 115 cases:<ref name=pmid22924843>{{Cite journal  | last1 = Shimizu-Kohno | first1 = K. | last2 = Kimura | first2 = Y. | last3 = Kiyasu | first3 = J. | last4 = Miyoshi | first4 = H. | last5 = Yoshida | first5 = M. | last6 = Ichikawa | first6 = R. | last7 = Niino | first7 = D. | last8 = Ohshima | first8 = K. | title = Malignant lymphoma of the spleen in Japan: a clinicopathological analysis of 115 cases. | journal = Pathol Int | volume = 62 | issue = 9 | pages = 577-82 | month = Sep | year = 2012 | doi = 10.1111/j.1440-1827.2012.02844.x | PMID = 22924843 }}</ref>
#[[Diffuse large B-cell lymphoma]] (DLBCL).
#[[Splenic marginal zone lymphoma]] (SMZL).<ref name=pmid20350661>{{Cite journal  | last1 = Bennett | first1 = M. | last2 = Schechter | first2 = GP. | title = Treatment of splenic marginal zone lymphoma: splenectomy versus rituximab. | journal = Semin Hematol | volume = 47 | issue = 2 | pages = 143-7 | month = Apr | year = 2010 | doi = 10.1053/j.seminhematol.2010.01.004 | PMID = 20350661 }}</ref>
#[[Follicular lymphoma]].
#Splenic B-cell lymphoma, unclassifiable.
#[[Peripheral T-cell lymphoma]], not otherwise specified.
Less common lymphoid neoplasms of the spleen:
*[[Hairy cell leukemia]].
*Splenic diffuse red pulp small B cell lymphoma.<ref name=pmid20677173>{{cite journal |author=Baseggio L, Traverse-Glehen A, Callet-Bauchu E, ''et al.'' |title=Relevance of a scoring system including CD11c expression in the identification of splenic diffuse red pulp small B-cell lymphoma (SRPL) |journal=Hematol Oncol |volume=29 |issue=1 |pages=47–51 |year=2011 |month=March |pmid=20677173 |doi=10.1002/hon.957 |url=}}</ref>


==DDx by compartment==
==DDx by compartment==
===White pulp malignant===
*[[Diffuse large B-cell lymphoma]].
*[[Splenic marginal zone lymphoma]].
*[[Follicular lymphoma]].
===Red pulp benign===
===Red pulp benign===
*[[Polycythemia rubra vera]] (AKA polycythemia vera).
*[[Polycythemia rubra vera]] (AKA polycythemia vera).
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**[[Budd-Chiari syndrome]] (AKA hepatic vein obstruction).
**[[Budd-Chiari syndrome]] (AKA hepatic vein obstruction).
**Splenic vein thrombosis.
**Splenic vein thrombosis.
*Congestive heart failure.
*[[Congestive heart failure]].
*[[Acute splenitis]].


===Red pulp malignant===
===Red pulp malignant===
*[[Hairy cell leukemia]].
*[[Hairy cell leukemia]].
*Acute leukemia.<ref name=Ref_WMSP_596>{{Ref WMSP|596}}</ref>
*Acute leukemia.<ref name=Ref_WMSP_596>{{Ref WMSP|596}}</ref>
*[[Hepatosplenic T-cell lymphoma]]<ref name=Ref_WMSP595>{{Ref WMSP|595}}</ref> (AKA Hepatosplenic γδ T-cell lymphoma).
*[[Hepatosplenic T-cell lymphoma]]<ref name=Ref_WMSP595>{{Ref WMSP|595}}</ref> (e.g. Hepatosplenic γδ T-cell lymphoma).


=Specific disorders=
=Specific disorders=
==Hyaloserositis==
==Splenic laceration==
*Properly referred to as ''hyaloserositis of the spleen''.
{{Main|Splenic laceration}}
*Capsule of the spleen is white - resembles sugar-coating; also known as ''sugar-coated spleen''.<ref>URL: [http://www.drugs.com/dict/sugar-coated-spleen.html http://www.drugs.com/dict/sugar-coated-spleen.html]. Accessed on: 1 September 2010.</ref>
 
*Importance: ''none'' - benign.
==Hyaloserositis of the spleen==
*[[AKA]] ''sugar-coated spleen''.
===General===
*Benign.
*Common [[autopsy]] finding.
 
===Gross===
*Capsule of the spleen is white - resembles sugar-coating.<ref>URL: [http://www.drugs.com/dict/sugar-coated-spleen.html http://www.drugs.com/dict/sugar-coated-spleen.html]. Accessed on: 1 September 2010.</ref>
 
===Microscopic===
Features:
*Hyaline material adherent to splenic capsule.
**Hyaline material = pink acellular crap on a [[H&E stain]].


Image(s):
====Images====
*[http://en.wikipedia.org/wiki/File:Spleen_hyaloserositis_-_intermed_mag.jpg Hyaloserositis (WC)].
<gallery>
Image:Spleen_hyaloserositis_-_intermed_mag.jpg | Hyaloserositis - intermed. mag. (WC)
Image:Spleen_hyaloserositis_-b-_high_mag.jpg | Hyaloserositis - high mag. (WC)
</gallery>


==Mononucleosis==
==Mononucleosis==
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**Cells indented by adjacent RBCs on blood smears.<ref name=utah_endo>URL: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/hpfrm.html http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/hpfrm.html]. Accessed on: 4 December 2011.</ref>
**Cells indented by adjacent RBCs on blood smears.<ref name=utah_endo>URL: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/hpfrm.html http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/hpfrm.html]. Accessed on: 4 December 2011.</ref>


Image:
Images:
[http://library.med.utah.edu/WebPath/jpeg4/ENDO033.jpg Mononucleosis (med.utah.edu)].<ref name=utah_endo>URL: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/hpfrm.html http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/hpfrm.html]. Accessed on: 4 December 2011.</ref>
*[http://library.med.utah.edu/WebPath/jpeg5/HEME013.jpg Mononucleosis (med.utah.edu)].<ref name=utah_endo>URL: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/hpfrm.html http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/hpfrm.html]. Accessed on: 4 December 2011.</ref>
*[http://path.upmc.edu/cases/case37.html Mononucleosis - several images (upmc.edu)].
 
===Flow cytometry===
*CD8 >>> CD4.<ref>URL: [http://path.upmc.edu/cases/case37/gross.html http://path.upmc.edu/cases/case37/gross.html]. Accessed on: 2 January 2012.</ref>


==Littoral cell angioma==
==Littoral cell angioma==
:For ''angioma'' see ''[[vascular malformations]]''.
:For ''angioma'' see ''[[vascular malformations]]''.
*[[AKA]] ''littoral cell angioma of the spleen''.
{{Main|Littoral cell angioma}}
===General===
Features:<ref name=pmid15329769>{{Cite journal  | last1 = Tan | first1 = YM. | last2 = Chuah | first2 = KL. | last3 = Wong | first3 = WK. | title = Littoral cell angioma of the spleen. | journal = Ann Acad Med Singapore | volume = 33 | issue = 4 | pages = 524-6 | month = Jul | year = 2004 | doi =  | PMID = 15329769 | url=http://www.annals.edu.sg/pdf200408/V33N4p524.pdf }}</ref>
*Rare.
*Benign.


Clinical:<ref name=pmid15329769/><ref name=pmid11547761>{{Cite journal  | last1 = Dascalescu | first1 = CM. | last2 = Wendum | first2 = D. | last3 = Gorin | first3 = NC. | title = Littoral-cell angioma as a cause of splenomegaly. | journal = N Engl J Med | volume = 345 | issue = 10 | pages = 772-3 | month = Sep | year = 2001 | doi = 10.1056/NEJM200109063451016 | PMID = 11547761 | url=http://www.nejm.org/doi/full/10.1056/NEJM200109063451016 }}</ref>
==Splenic hamartoma==
*Pyrexia (fever), splenomegaly, weight loss, solitary hypodense lesion on imaging.
{{Main|Splenic hamartoma}}


Note:
==Splenic infarct==
*''Littoral cells'' = line vascular channels of the red pulp.<ref name=pmid15329769/>
*[[AKA]] ''splenic [[infarct]]ion''.
**''Littoral'' = related to ''shore''.<ref>URL: [http://www.merriam-webster.com/dictionary/littoral http://www.merriam-webster.com/dictionary/littoral]. Accessed on: 24 July 2011.</ref><ref>URL: [http://dictionary.reference.com/browse/littoral http://dictionary.reference.com/browse/littoral]. Accessed on: 24 July 2011.</ref>
*[[AKA]] ''infarction of the spleen''.


===Microscopic===
===General===
Features:<ref name=pmid15329769/>
Classic textbook causes:<ref name=pmid20928991>{{Cite journal  | last1 = Lawrence | first1 = YR. | last2 = Pokroy | first2 = R. | last3 = Berlowitz | first3 = D. | last4 = Aharoni | first4 = D. | last5 = Hain | first5 = D. | last6 = Breuer | first6 = GS. | last7 = Osler | first7 = W. | title = Splenic infarction: an update on William Osler's observations. | journal = Isr Med Assoc J | volume = 12 | issue = 6 | pages = 362-5 | month = Jun | year = 2010 | doi =  | PMID = 20928991 | URL = http://www.ima.org.il/imaj/dynamic/web/ArtFromPubmed.asp?year=2010&month=06&page=362 }}</ref>
*Anastoming, small vascular channels.
*Septic embolus due to [[bacterial endocarditis]].
*Cystic spaces.  
*[[Sickle cell disease]].
**May have papillary projections.<ref name=pmid11547761/>
 
DDx:<ref name=pmid15329769/>
*Vascular lesions:
**[[Hemangioendothelioma]].
**[[Angiosarcoma]].
**[[Hemangiopericytoma]].
**[[Splenic hamartoma]].


Images:
Usual causes:<ref name=pmid20928991/>
*[http://www.webpathology.com/image.asp?n=5&Case=382 LCA - low mag. (webpathology.com)].
*Hematologic malignancy.
*[http://www.webpathology.com/image.asp?n=6&Case=382 LCA - high mag. (webpathology.com)].
*Intracardiac thrombus.
*[http://commons.wikimedia.org/wiki/File:Littoral_cell_angioma_of_the_spleen_-_very_low_mag.jpg LCA - very low mag. (WC)].
*Bacterial endocarditis.
*[http://commons.wikimedia.org/wiki/File:Littoral_cell_angioma_of_the_spleen_-_intermed_mag.jpg LCA - intermed. mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Littoral_cell_angioma_of_the_spleen_-_very_high_mag.jpg LCA - very high mag. (WC)].


===IHC===
Clinical:<ref name=pmid20928991/>
Features:<ref name=pmid15329769/>
*Left upper quadrant pain ~ 1/3 of cases.
*CD68 +ve.
*Fever ~ 1/3 of cases.
*CD31 +ve
*Leukocytosis ~ 1/2 of cases.


==Splenic hamartoma==
===Gross===
===General===
*Classically wedge-shaped; triangular on section.
*Benign.
**The base of the triangle runs along the surface.
*Women > men.<ref name=pmid21393931>{{Cite journal  | last1 = Sankar | first1 = S. | last2 = Thanka | first2 = J. | last3 = Jagdishchandrabose | first3 = S. | last4 = Rajendran | first4 = S. | title = Splenic hamartoma: a rare vascular space occupying lesion of the spleen. | journal = Indian J Pathol Microbiol | volume = 54 | issue = 1 | pages = 223-5 | month =  | year =  | doi = 10.4103/0377-4929.77416 | PMID = 21393931 }}</ref>
**The apex points to the obstructed vessel that lead to the infarct.


===Microscopic===
===Microscopic===
Features:<ref name=pmid19123729/>
:''See [[necrosis]]''.
*Variable size irregular vascular channels
*Red pulp-like disorganized stroma
*+/-Lymphoid follicles.
 
===IHC===
Features:<ref name=pmid19123729>{{Cite journal  | last1 = Lee | first1 = H. | last2 = Maeda | first2 = K. | title = Hamartoma of the spleen. | journal = Arch Pathol Lab Med | volume = 133 | issue = 1 | pages = 147-51 | month = Jan | year = 2009 | doi = 10.1043/1543-2165-133.1.147 | PMID = 19123729 }}
</ref>
*CD8 +ve endothelial cells.


=Weird stuff=
=Weird stuff=
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==Follicular dendritic cell tumour==
==Follicular dendritic cell tumour==
*[[AKA]] ''follicular dendritic cell sarcoma'',<ref name=pmid17515347>{{Cite journal  | last1 = Leipsic | first1 = JA. | last2 = McAdams | first2 = HP. | last3 = Sporn | first3 = TA. | title = Follicular dendritic cell sarcoma of the mediastinum. | journal = AJR Am J Roentgenol | volume = 188 | issue = 6 | pages = W554-6 | month = Jun | year = 2007 | doi = 10.2214/AJR.04.1530 | PMID = 17515347 }}</ref> ''dendritic reticulum cell sarcoma'', (???) ''follicular dendritic cell neoplasm''. (???)
*[[AKA]] ''follicular dendritic cell sarcoma''.
===General===
{{Main|Follicular dendritic cell sarcoma}}
*Very rare.
*Behave like a low-grade sarcoma.<ref name=pmid9606805>{{Cite journal  | last1 = Perez-Ordoñez | first1 = B. | last2 = Rosai | first2 = J. | title = Follicular dendritic cell tumor: review of the entity. | journal = Semin Diagn Pathol | volume = 15 | issue = 2 | pages = 144-54 | month = May | year = 1998 | doi =  | PMID = 9606805 }}</ref>
*Reported association with [[Castleman disease]] (hyaline-vascular type).<ref name=pmid9606805/>
 
===Microscopic===
Features:<ref name=pmid9606805/>
*Oval or spindle-shaped cellular & nuclear morphology.
*Variable architecture (sheets, fascicles, whorles, storiform pattern).
*Nuclei:
**Small nucleoli.
**Clear or dispersed chromatin.
*Multinucleated cells.
*Interspersed small lymphocytes - '''distinctive feature'''.
*+/-Necrosis.
*+/-Marked nuclear atypia.
*+/-Abundant mitoses.
 
Images:
*[http://www.ajronline.org/content/188/6/W554/F7.expansion FDCS (ajronline.org)].<ref name=pmid17515347/>
*[http://www.webpathology.com/image.asp?case=391&n=1 DRCS (webpathology.com)].
 
===IHC===
Features:<ref name=pmid9606805/>
*CD21 +ve.
*CD35 +ve.
*Ki-M4p +ve
*Ki-FDRC1p +ve.
*Vimentin +ve.
 
*S-100 +ve/-ve.
*Muscle-specific actin +ve/-ve.
*EMA +ve/-ve.


==Hepatosplenic T-cell lymphoma==
==Hepatosplenic T-cell lymphoma==
*[[AKA]] ''hepatosplenic γδ T-cell lymphoma''.
*Abbreviated ''HSTL''.
*Abbreviated ''HSTL''.
===General===
===General===
*Rare.
*Rare.
*Prognosis: poor.
Subtypes:<ref name=webpath_HSTL/>
#''Hepatosplenic γδ T-cell lymphoma''.
#*May be written ''Hepatosplenic gamma/delta T-cell lymphoma''.
#''Hepatosplenic αβ T-cell lymphoma''.
#*May be written ''Hepatosplenic alpha/beta T-cell lymphoma''.


Clinical triad:
Clinical triad:
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===Microscopic===
===Microscopic===
Features:<ref name=Ref_WMSP595>{{Ref WMSP|595}}</ref>
Features:<ref name=Ref_WMSP595>{{Ref WMSP|595}}</ref><ref name=webpath_HSTL>URL: [http://www.webpathology.com/image.asp?case=378&n=15 http://www.webpathology.com/image.asp?case=378&n=15]. Accessed on: 22 December 2011.</ref>
*Small cell lymphoma.
*Small cell lymphoma/Intermediate cell lymphoma.
**+/-"Folded" nuclei.


DDx:
DDx:
*[[Hairy cell leukemia]].
*[[Hairy cell leukemia]].
Images:
*[http://www.webpathology.com/image.asp?n=14&Case=378 HSTL - low mag. (webpathology.com)].
*[http://www.webpathology.com/image.asp?case=378&n=15 HSTL - high mag. (webpathology.com)].


===IHC===
===IHC===
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*CD4 -ve.
*CD4 -ve.
*CD8 -ve.
*CD8 -ve.
*NK cell-associated antigens +ve:<ref name=pmid9191001/>
**[[CD56]],<ref name=pmid15458516>{{Cite journal  | last1 = Niitsu | first1 = N. | last2 = Kohri | first2 = M. | last3 = Togano | first3 = T. | last4 = Nakamine | first4 = H. | last5 = Nakamura | first5 = S. | last6 = Iwabuchi | first6 = K. | last7 = Higashihara | first7 = M. | title = Development of hepatosplenic gammadelta T-cell lymphoma with pancytopenia during early pregnancy: a case report and review of the literature. | journal = Eur J Haematol | volume = 73 | issue = 5 | pages = 367-71 | month = Nov | year = 2004 | doi = 10.1111/j.1600-0609.2004.00300.x | PMID = 15458516 }}</ref> CD11c, CD16.
*Effector proteins +ve.<ref name=pmid9191001>{{Cite journal  | last1 = Salhany | first1 = KE. | last2 = Feldman | first2 = M. | last3 = Kahn | first3 = MJ. | last4 = Peritt | first4 = D. | last5 = Schretzenmair | first5 = RD. | last6 = Wilson | first6 = DM. | last7 = DiPaola | first7 = RS. | last8 = Glick | first8 = AD. | last9 = Kant | first9 = JA. | title = Hepatosplenic gammadelta T-cell lymphoma: ultrastructural, immunophenotypic, and functional evidence for cytotoxic T lymphocyte differentiation. | journal = Hum Pathol | volume = 28 | issue = 6 | pages = 674-85 | month = Jun | year = 1997 | doi =  | PMID = 9191001 }}</ref>
**Perforin, granzyme B, TIA-1, Fas ligand.
==Myelolipoma of the spleen==
{{Main|Myelolipoma}}
==Immune thrombocytopenic purpura==
{{Main|Immune thrombocytopenic purpura}}
Spleens are removed for this.
==Splenogonadal fusion==
{{Main|Splenogonadal fusion}}


=See also=
=See also=
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[[Category:Haematopathology]]
[[Category:Haematopathology]]
[[Category:Spleen]]
Michael
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