Difference between revisions of "Spleen"

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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===
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=Specific disorders=
=Specific disorders=
==Splenic laceration==
{{Main|Splenic laceration}}
==Hyaloserositis of the spleen==
==Hyaloserositis of the spleen==
*[[AKA]] ''sugar-coated spleen''.
*[[AKA]] ''sugar-coated spleen''.
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**Hyaline material = pink acellular crap on a [[H&E stain]].
**Hyaline material = pink acellular crap on a [[H&E stain]].


Images:
====Images====
*[http://commons.wikimedia.org/wiki/File:Spleen_hyaloserositis_-_intermed_mag.jpg Hyaloserositis - intermed. mag. (WC)].
<gallery>
*[http://commons.wikimedia.org/wiki/File:Spleen_hyaloserositis_-b-_high_mag.jpg Hyaloserositis - high mag. (WC)]
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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==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/>
Usual causes:<ref name=pmid20928991/>
*Vascular lesions:
*Hematologic malignancy.
**[[Hemangioendothelioma]].
*Intracardiac thrombus.
**[[Angiosarcoma]].
*Bacterial endocarditis.
**[[Hemangiopericytoma]].
**[[Splenic hamartoma]].


Images:
Clinical:<ref name=pmid20928991/>
*[http://www.webpathology.com/image.asp?n=5&Case=382 LCA - low mag. (webpathology.com)].
*Left upper quadrant pain ~ 1/3 of cases.
*[http://www.webpathology.com/image.asp?n=6&Case=382 LCA - high mag. (webpathology.com)].
*Fever ~ 1/3 of cases.
*[http://commons.wikimedia.org/wiki/File:Littoral_cell_angioma_of_the_spleen_-_very_low_mag.jpg LCA - very low mag. (WC)].
*Leukocytosis ~ 1/2 of cases.
*[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===
===Gross===
Features:<ref name=pmid15329769/>
*Classically wedge-shaped; triangular on section.
*CD68 +ve.
**The base of the triangle runs along the surface.
*CD31 +ve
**The apex points to the obstructed vessel that lead to the infarct.
 
==Splenic hamartoma==
===General===
*Benign.
*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>


===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==
*Abbreviated ''FDCT''.
*[[AKA]] ''follicular dendritic cell sarcoma''.
*[[AKA]] ''follicular dendritic cell sarcoma'' (abbreviated ''FDCS''),<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''. (???)
{{Main|Follicular dendritic cell sarcoma}}
===General===
*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)].
*[http://path.upmc.edu/cases/case55/dx.html FDCT - several crappy images (upmc.edu)].
 
===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==
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*CD8 -ve.
*CD8 -ve.
*NK cell-associated antigens +ve:<ref name=pmid9191001/>
*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.
**[[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>
*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.
**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]]

Latest revision as of 12:50, 7 September 2016

Spleen.

The spleen is a forgotten organ.

Normal histology

  • Capsule.
  • Red pulp - red blood cells.
  • White pulp - white blood cells.
  • Marginal zone - between red pulp and white pulp.[1]

Images

  • Normal spleen (left) and splenic hamartoma (right). H&E stain. (WC)

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

Terminology Etiology Description Other
Ruptured spleen trauma hemorrhagic, capsule disrupted possible assocations mononucleosis, medical procedure - esp. colonoscopy,[2] others
"Lardaceous spleen"[3] 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:

Splenic enlargement

Splenomegaly redirects here.

Lymphoid neoplasms of the spleen

Lymphomas of the spleen in order of prevalence - in a series of 115 cases:[5]

  1. Diffuse large B-cell lymphoma (DLBCL).
  2. Splenic marginal zone lymphoma (SMZL).[6]
  3. Follicular lymphoma.
  4. Splenic B-cell lymphoma, unclassifiable.
  5. Peripheral T-cell lymphoma, not otherwise specified.

Less common lymphoid neoplasms of the spleen:

DDx by compartment

White pulp malignant

Red pulp benign

Red pulp malignant

Specific disorders

Splenic laceration

Main article: Splenic laceration

Hyaloserositis of the spleen

  • AKA sugar-coated spleen.

General

Gross

  • Capsule of the spleen is white - resembles sugar-coating.[10]

Microscopic

Features:

  • Hyaline material adherent to splenic capsule.
    • Hyaline material = pink acellular crap on a H&E stain.

Images

  • Hyaloserositis - intermed. mag. (WC)

  • Hyaloserositis - high mag. (WC)

Mononucleosis

General

  • EBV infection.
  • +/-Massive splenic enlargement.

Clinical:

  • Monospot test +ve.

Microscopic

Features:

  • Atypical lymphoid cells.
    • Abundant basophilic cytoplasm.
    • Cells indented by adjacent RBCs on blood smears.[11]

Images:

Flow cytometry

Littoral cell angioma

For angioma see vascular malformations.
Main article: Littoral cell angioma

Splenic hamartoma

Main article: Splenic hamartoma

Splenic infarct

General

Classic textbook causes:[13]

Usual causes:[13]

  • Hematologic malignancy.
  • Intracardiac thrombus.
  • Bacterial endocarditis.

Clinical:[13]

  • Left upper quadrant pain ~ 1/3 of cases.
  • Fever ~ 1/3 of cases.
  • Leukocytosis ~ 1/2 of cases.

Gross

  • Classically wedge-shaped; triangular on section.
    • The base of the triangle runs along the surface.
    • The apex points to the obstructed vessel that lead to the infarct.

Microscopic

See necrosis.

Weird stuff

  • Dendritic cell tumours.[8]
    • Interdigitating dendritic cell tumour.
    • Follicular dendritic cell tumour.

Follicular dendritic cell tumour

  • AKA follicular dendritic cell sarcoma.
Main article: Follicular dendritic cell sarcoma

Hepatosplenic T-cell lymphoma

  • Abbreviated HSTL.

General

  • Rare.
  • Prognosis: poor.

Subtypes:[14]

  1. Hepatosplenic γδ T-cell lymphoma.
    • May be written Hepatosplenic gamma/delta T-cell lymphoma.
  2. Hepatosplenic αβ T-cell lymphoma.
    • May be written Hepatosplenic alpha/beta T-cell lymphoma.

Clinical triad:

  1. Hepatosplenomegaly.
  2. Cytopenias (anemia, thrombocytopenia).
  3. Sinusoidal tropism.

Microscopic

Features:[9][14]

  • Small cell lymphoma/Intermediate cell lymphoma.
    • +/-"Folded" nuclei.

DDx:

Images:

IHC

Features:[9]

  • CD4 -ve.
  • CD8 -ve.
  • NK cell-associated antigens +ve:[15]
  • Effector proteins +ve.[15]
    • Perforin, granzyme B, TIA-1, Fas ligand.

Myelolipoma of the spleen

Main article: Myelolipoma

Immune thrombocytopenic purpura

Main article: Immune thrombocytopenic purpura

Spleens are removed for this.

Splenogonadal fusion

Main article: Splenogonadal fusion

See also

References

  1. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 589. ISBN 978-0781765275.
  2. ↑ Aubrey-Bassler, FK.; Sowers, N. (Aug 2012). "613 cases of splenic rupture without risk factors or previously diagnosed disease: a systematic review.". BMC Emerg Med 12 (1): 11. doi:10.1186/1471-227X-12-11. PMID 22889306.
  3. ↑ Klatt, Edward C. (2006). Robbins and Cotran Atlas of Pathology (1st ed.). Saunders. pp. 91. ISBN 978-1416002741.
  4. ↑ Maakaroun, NR.; Moanna, A.; Jacob, JT.; Albrecht, H. (Mar 2010). "Viral infections associated with haemophagocytic syndrome.". Rev Med Virol 20 (2): 93-105. doi:10.1002/rmv.638. PMID 20127750.
  5. ↑ Shimizu-Kohno, K.; Kimura, Y.; Kiyasu, J.; Miyoshi, H.; Yoshida, M.; Ichikawa, R.; Niino, D.; Ohshima, K. (Sep 2012). "Malignant lymphoma of the spleen in Japan: a clinicopathological analysis of 115 cases.". Pathol Int 62 (9): 577-82. doi:10.1111/j.1440-1827.2012.02844.x. PMID 22924843.
  6. ↑ Bennett, M.; Schechter, GP. (Apr 2010). "Treatment of splenic marginal zone lymphoma: splenectomy versus rituximab.". Semin Hematol 47 (2): 143-7. doi:10.1053/j.seminhematol.2010.01.004. PMID 20350661.
  7. ↑ Baseggio L, Traverse-Glehen A, Callet-Bauchu E, et al. (March 2011). "Relevance of a scoring system including CD11c expression in the identification of splenic diffuse red pulp small B-cell lymphoma (SRPL)". Hematol Oncol 29 (1): 47–51. doi:10.1002/hon.957. PMID 20677173.
  8. ↑ 8.0 8.1 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 596. ISBN 978-0781765275.
  9. ↑ 9.0 9.1 9.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 595. ISBN 978-0781765275.
  10. ↑ URL: http://www.drugs.com/dict/sugar-coated-spleen.html. Accessed on: 1 September 2010.
  11. ↑ 11.0 11.1 URL: http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/hpfrm.html. Accessed on: 4 December 2011.
  12. ↑ URL: http://path.upmc.edu/cases/case37/gross.html. Accessed on: 2 January 2012.
  13. ↑ 13.0 13.1 13.2 Lawrence, YR.; Pokroy, R.; Berlowitz, D.; Aharoni, D.; Hain, D.; Breuer, GS.; Osler, W. (Jun 2010). "Splenic infarction: an update on William Osler's observations.". Isr Med Assoc J 12 (6): 362-5. PMID 20928991.
  14. ↑ 14.0 14.1 URL: http://www.webpathology.com/image.asp?case=378&n=15. Accessed on: 22 December 2011.
  15. ↑ 15.0 15.1 Salhany, KE.; Feldman, M.; Kahn, MJ.; Peritt, D.; Schretzenmair, RD.; Wilson, DM.; DiPaola, RS.; Glick, AD. et al. (Jun 1997). "Hepatosplenic gammadelta T-cell lymphoma: ultrastructural, immunophenotypic, and functional evidence for cytotoxic T lymphocyte differentiation.". Hum Pathol 28 (6): 674-85. PMID 9191001.
  16. ↑ Niitsu, N.; Kohri, M.; Togano, T.; Nakamine, H.; Nakamura, S.; Iwabuchi, K.; Higashihara, M. (Nov 2004). "Development of hepatosplenic gammadelta T-cell lymphoma with pancytopenia during early pregnancy: a case report and review of the literature.". Eur J Haematol 73 (5): 367-71. doi:10.1111/j.1600-0609.2004.00300.x. PMID 15458516.

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