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[[Image:Lymphoma macro.jpg | thumb | 200px | right | Lymphoma at [[cut-up]]. (WC/Emmanuelm)]]
'''Lymphoma''' is almost a specialty for itself.  It can be subclassified a number of ways.   
'''Lymphoma''' is almost a specialty for itself.  It can be subclassified a number of ways.   


This article is an introduction to ''lymphoma''.  An introduction to ''lymph nodes'' and ''lymph node pathology that is not lymphoma'' are in the articles ''[[lymph node]]'' and ''[[lymph node pathology]]''.
This article is an introduction to ''lymphoma''.  An introduction to ''lymph nodes'' and ''lymph node pathology that is not lymphoma'' are in the articles ''[[lymph node]]'' and ''[[lymph node pathology]]''.
A general introduction to haematopathology is in the ''[[haematopathology]]'' article.
==General==
The presentations are variable and similar to that of other malignancies. They may include:
*Mass effect.
*Weight loss.
*Fever.
*Night sweats.
*Infection.
*Incidental:
**Routine blood work for something unrelated.
**Life insurance work-up.
===B symptoms===
*May be seen in [[Hodgkin lymphoma]] and non-Hodgkin lymphoma.
*Presence correlates with higher stage.
*Predictor of poor prognosis independent of stage.
All of 'em are required to call "B symptoms"<ref name=pmid5121694>{{Cite journal  | last1 = Carbone | first1 = PP. | last2 = Kaplan | first2 = HS. | last3 = Musshoff | first3 = K. | last4 = Smithers | first4 = DW. | last5 = Tubiana | first5 = M. | title = Report of the Committee on Hodgkin's Disease Staging Classification. | journal = Cancer Res | volume = 31 | issue = 11 | pages = 1860-1 | month = Nov | year = 1971 | doi =  | PMID = 5121694 | URL = http://cancerres.aacrjournals.org/cgi/pmidlookup?view=long&pmid=5121694 }}</ref> - mnemonic ''These '''B'''othersome features cause '''W'''ednesday '''N'''ight '''F'''ever'':<ref>URL: [http://www.internalizemedicine.com/2011/12/bury-buzzword-b-symptoms.html http://www.internalizemedicine.com/2011/12/bury-buzzword-b-symptoms.html]. Accessed on: 28 March 2012.</ref>
*Weight loss - >10% in 6 months.
*Night sweats.
*Fever - 38 degree C that is unexplained.
Note:
*''A symptoms'' do '''not''' exist. The term comes from the staging system. In the "A" of the staging system the above symptoms are '''a'''bsent.


==Lymphoma classification==
==Lymphoma classification==
Lymphomas can be divided into:
Lymphomas can be divided into:
*Hodgkin's lymphoma.
*[[Hodgkin's lymphoma]].
*Non-Hodgkin's lymphoma (NHL).
*Non-Hodgkin's lymphoma (NHL).


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Two most common NHLs:
Two most common NHLs:
*Follicular lymphoma (FL).
*[[Follicular lymphoma]] (FL).
*Diffuse large B-cell lymphoma (DLBCL).
*[[Diffuse large B-cell lymphoma]] (DLBCL).


===Leukemia as a med student===
===Leukemia as a med student===
*Acute lymphoid leukemia (ALL) - predominantly in '''smALL''' people, i.e. children.
*Acute lymphoid leukemia (ALL) - predominantly in '''smALL''' people, i.e. children.
*Acute myeloid leukemia (AML).
*[[Acute myeloid leukemia]] (AML).
*Chronic myeloid leukemia (CML).
*[[Chronic myeloid leukemia]] (CML).
*Chronic lymphoid leukemia (CLL) - relatively good prognosis.
*[[Chronic lymphocytic leukemia]] (CLL) - relatively good prognosis.


===Histologic classification===
===Histologic classification===
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===Histologic terms===
====Histologic terms====
*Lymphomas = cells look discohesive, may be difficult to differentiate from poor differentiated carcinoma.
*Lymphomas = cells look discohesive, may be difficult to differentiate from poor differentiated carcinoma.
*Auer rods = Acute myeloid leukemia.
*[[Auer rods]] = [[acute myeloid leukemia]].
**Granular cytoplasmic rod (0.5-1 x4-6 micrometres).
**Granular cytoplasmic rod (0.5-1 x4-6 micrometres).
*Reed-Sternberg cells = Hodgkin's lymphoma.
**Not pathognomonic.
*Reed-Sternberg cells = [[Hodgkin's lymphoma]].
**Large cell - very large nucleus.
**Large cell - very large nucleus.
***Classically binucleated.
***Classically binucleated.
*Russell bodies = Plasmacytoma (+others).
*Russell bodies = [[plasmacytoma]] (+others).
**Eosinophilic, large, homogenous immunoglobulin-containing inclusions.<ref name=pmid3979421>{{cite journal |author=Alanen A, Pira U, Lassila O, Roth J, Franklin RM |title=Mott cells are plasma cells defective in immunoglobulin secretion |journal=Eur. J. Immunol. |volume=15 |issue=3 |pages=235–42 |year=1985 |month=March |pmid=3979421 |doi= |url=}}</ref>
**Eosinophilic, large, homogenous immunoglobulin-containing inclusions.<ref name=pmid3979421>{{cite journal |author=Alanen A, Pira U, Lassila O, Roth J, Franklin RM |title=Mott cells are plasma cells defective in immunoglobulin secretion |journal=Eur. J. Immunol. |volume=15 |issue=3 |pages=235–42 |year=1985 |month=March |pmid=3979421 |doi= |url=}}</ref>
***''Mott cell'' is a cell that contains Russell bodies.<ref name=pmid3979421/>
***''Mott cell'' is a cell that contains Russell bodies.<ref name=pmid3979421/>
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**AKA ''common lymphocyte antigen''.
**AKA ''common lymphocyte antigen''.
**Useful to differentiate from carcinomas (e.g. small cell carcinoma).
**Useful to differentiate from carcinomas (e.g. small cell carcinoma).
Others:
*[[AE1/AE3]] -ve -- to r/o carcinoma.
====T cell markers====
====T cell markers====
*CD2 -- T cell marker (all T cells).
*CD2 -- T cell marker (all T cells).
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*CD43 -- +ve in mantle cell lymphoma
*CD43 -- +ve in mantle cell lymphoma


*Alk1.
*ALK1<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/601284 http://www.ncbi.nlm.nih.gov/omim/601284]. Accessed on: 31 August 2010.</ref> - prognostic in anaplastic large cell lymphoma (ALCL).<ref name=pmid9250148>{{cite journal |author=Pittaluga S, Wlodarska I, Pulford K, ''et al.'' |title=The monoclonal antibody ALK1 identifies a distinct morphological subtype of anaplastic large cell lymphoma associated with 2p23/ALK rearrangements |journal=Am. J. Pathol. |volume=151 |issue=2 |pages=343–51 |year=1997 |month=August |pmid=9250148 |pmc=1858018 |doi= |url=}}</ref>
*TIA1.<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/603518 http://www.ncbi.nlm.nih.gov/omim/603518]. Accessed on: 18 August 2010.</ref>
*TIA1 - cytotoxic T-cell.<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/603518 http://www.ncbi.nlm.nih.gov/omim/603518]. Accessed on: 18 August 2010.</ref>
*Granzyme B.
*Granzyme B.
====B cell markers====
====B cell markers====
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*CD10 -- follicle center.
*CD10 -- follicle center.
*BCL6 -- follicle center.
*BCL6 -- follicle center.
*BCL2 -- small B cell lymphomas; T cell marker.
*BCL2 -- normally negative in germinal centers (GCs), marks T cell; +ve in small B cell lymphomas.
*MUM1.
*MUM1 -- -ve in follicular lymphoma, +ve in DLBCL that did not arise from follicular lymphoma.
 
====Plasma cell====
*Kappa -- usu. slightly stronger than lambda.
*Lambda.
*CD56<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/116930 http://www.ncbi.nlm.nih.gov/omim/116930]. Accessed on: 31 August 2010.</ref> -- also +ve in NK/T cell lymphomas.
*CD57 -- +ve in [[T-cell large granular lymphocytic leukemia]].<ref>URL: [http://www.nature.com/bmt/journal/v33/n1/full/1704298a.html http://www.nature.com/bmt/journal/v33/n1/full/1704298a.html]. Accessed on: 31 August 2010.</ref>.
*CD138.


====Follicular dendritic cells====
====Follicular dendritic cells====
*CD23 -- follicular dendritic cells.
*CD23 -- follicular dendritic cells.
*CD21 -- follicular dendritic cells.
*CD21 -- follicular dendritic cells, considered more sensitive than CD23.<ref name=pmid16280657>{{Cite journal  | last1 = Troxell | first1 = ML. | last2 = Schwartz | first2 = EJ. | last3 = van de Rijn | first3 = M. | last4 = Ross | first4 = DT. | last5 = Warnke | first5 = RA. | last6 = Higgins | first6 = JP. | last7 = Natkunam | first7 = Y. | title = Follicular dendritic cell immunohistochemical markers in angioimmunoblastic T-cell lymphoma. | journal = Appl Immunohistochem Mol Morphol | volume = 13 | issue = 4 | pages = 297-303 | month = Dec | year = 2005 | doi =  | PMID = 16280657 }}</ref>


====Hodgkin's lymphoma====
====Hodgkin's lymphoma====
=====Classic=====
=====Classic=====
*CD30 -- Hodgkin's lymphoma (most sensitive).
*CD30 +ve -- Hodgkin's lymphoma (most sensitive).
*CD15.
*CD15 +ve.
*PAX5 +ve.
*EMA -ve.
*EBER +ve/-ve.
 
Others:
*CD20, CD45: weak +ve or -ve.
 
=====NLPHL=====
*CD20 +ve.
*CD30 -ve, CD15 -ve.
*EMA +ve/-ve.
*EBER -ve.
 
====Others====
*Myeloperoxidase - in PMNs.
*Glycophorin C.
*CD61 -- megakaryocytes.
*TdT.
*CD34.
 
===Molecular pathology===
{{Main|Molecular pathology}}
{{Main|Molecular pathology tests}}
*T cell clonality study.
*B cell clonality study.
 
====Chromosomal translocations====
{{Main|Chromosomal_translocations#Lymphoma}}


==Hodgkin's lymphoma==
==Hodgkin's lymphoma==
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#*T lymphocytes only (no mix of cells).
#*T lymphocytes only (no mix of cells).
#Lymphocyte-depleted CHL - rare.
#Lymphocyte-depleted CHL - rare.
#*May be associated with HIV infection.<ref name=pmid20138008/>
#*May be associated with [[HIV]] infection.<ref name=pmid20138008/>


Memory device:  
Memory device:  
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*[http://commons.wikimedia.org/wiki/File:Popcorn_cell_in_nodular_lymphocyte_predominant_Hodgkin_lymphoma_-_very_high_mag_cropped.jpg Popcorn cell (WC)].
*[http://commons.wikimedia.org/wiki/File:Popcorn_cell_in_nodular_lymphocyte_predominant_Hodgkin_lymphoma_-_very_high_mag_cropped.jpg Popcorn cell (WC)].


==Follicular lymphoma==
==Small cell lymphomas==
{{main|Small cell lymphomas}}
{{main|Small cell lymphomas}}
This grouping includes:
*Follicular lymphoma.
*Marginal zone lymphoma.
**Nodal marginal zone lymphoma.
**Extranodal marginal zone lymphoma (MALT lymphoma).
**Splenic marginal zone lymphoma.
*Mantle zone lymphoma.
*Small lymphocytic lymphoma (SLL) / chronic lymphocytic leukemia (CLL).
*Hairy cell leukemia.


==Diffuse large B-cell lymphoma==
==Diffuse large B-cell lymphoma==
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Notes:
Notes:
*Large bizarre cells can occasionally mimic Reed-Sternberg cells, seen in [[Hodgkin lymphoma]].
*Large bizarre cells can occasionally mimic Reed-Sternberg cells, seen in [[Hodgkin lymphoma]].
==Intravascular lymphoma==
*[[AKA]] ''angiotropic lymphoma'', ''intravascular malignant lymphomatosis'', ''malignant angioendotheliomatosis''
*Rare.
*Usually B-cell lineage (see ''[[intravascular large B-cell lymphoma]]'').
**May be T-cell lineage.<ref name=pmid20337769>{{cite journal |author=Wang L, Li C, Gao T |title=Cutaneous intravascular anaplastic large cell lymphoma |journal=J Cutan Pathol |volume= |issue= |pages= |year=2010 |month=March |pmid=20337769 |doi=10.1111/j.1600-0560.2010.01538.x |url=}}</ref>


==Burkitt's lymphoma==
==Burkitt's lymphoma==
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Image: [http://en.wikipedia.org/wiki/File:Burkitt_lymphoma,_H%26E.jpg Starry-sky pattern - Ed Uthman (WC)].
Image: [http://en.wikipedia.org/wiki/File:Burkitt_lymphoma,_H%26E.jpg Starry-sky pattern - Ed Uthman (WC)].


==Plasmacytoma==
==Plasma cell neoplasms==
===General===
{{Main|Plasma cell neoplasms}}
*[[AKA]] ''plasma cell myleoma''.
*These arise from ''plasma cells''.
*Malignancy derived from the plasma cells.
*[[AKA]] ''plasma cell myeloma'', ''plasmacytoma''.
*Histologic component of ''multiple myeloma''; to diagnose multiple myeloma other (non-pathology) criteria are needed.
*''Multiple myeloma'' (a clinical diagnosis) fits into this category.
*Prognosis: poor.
 
===Microscopic===
Features:
*Abundant eosinophilic cytoplasm.
*Eccentrically placed nucleus.
**Usually with "clock face" morphology.
***"Clock face" morphology = chromatin clumps around the edge of the nucleus, like the numbers on a clock face.
**May have nucleoli.
*Russell bodies:
**Eosinophilic, large (10-15 micrometres), homogenous immunoglobulin-containing inclusions.
***Images: [http://commons.wikimedia.org/wiki/File:Russell_bodies_2_high_mag_mini.jpg Russell bodies (WC)], [http://www.healthsystem.virginia.edu/internet/hematology/hessimages/russell-bodies-website-arrow.jpg Russell bodies (healthsystem.virginia.edu)], [http://www.pathguy.com/lectures/russ2.jpg Russell bodies - several in one cell (pathguy.com)].
*Dutcher bodies - intranuclear crystalline rods.
**Dutcher bodies are ''PAS stain'' +ve.<ref>URL: [http://www.thefreelibrary.com/Dutcher+bodies+in+chronic+synovitis-a083551789 http://www.thefreelibrary.com/Dutcher+bodies+in+chronic+synovitis-a083551789]. Accessed on: 4 August 2010.</ref>
**Image [http://ashimagebank.hematologylibrary.org/cgi/content/full/2003/0227/100629 Dutcher bodies (hematologylibrary.org)].
*Prominent ''perinuclear hof'' - cytoplasmic crescent shaped lucency adjacent to the nuclear membrane (due to large Golgi apparatus); nucleus has a [http://en.wikipedia.org/wiki/Bib_%28garment%29 "bib"].
 
Images:
*[http://ashimagebank.hematologylibrary.org/cgi/content/full/2004/0126/100984 Various images (hematologylibrary.org)].
*[http://commons.wikimedia.org/wiki/File:Plasmacytoma1.jpg Plasmacytoma (WC)].
 
DDx:
*Neuroendocrine carcinoma - nucleus often has a plasmacytoid (plasma cell-like) appearance.


==Acute myeloid leukemia==
==Acute myeloid leukemia==
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===Complications===
===Complications===
*Chloroma - soft tissue mass.
*[[Chloroma]] - soft tissue mass.
*Leukostasis.
*Leukostasis.
**Occurs - lungs and brain.<ref>AML. Harrison's 16th Ed.</ref>
**Occurs - lungs and brain.<ref>AML. Harrison's 16th Ed.</ref>
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**Images: [http://en.wikipedia.org/wiki/File:Auer_rods.PNG Auer rods (WP)], [http://www.healthsystem.virginia.edu/internet/hematology/HessImages/Acute-myelogenous-leukemia-M4-100x-Auer-rods-website-arrow.jpg Auer rods (virginia.edu)].
**Images: [http://en.wikipedia.org/wiki/File:Auer_rods.PNG Auer rods (WP)], [http://www.healthsystem.virginia.edu/internet/hematology/HessImages/Acute-myelogenous-leukemia-M4-100x-Auer-rods-website-arrow.jpg Auer rods (virginia.edu)].


==Enteropathy-associated T cell lymphoma==
==Enteropathy-associated T-cell lymphoma==
===General===
*Abbreviated ''EATL''.
*Abbreviated ''EATL''.
*[[AKA]] ''enteropathy-type T cell lymphoma'' (ETTL).
*[[AKA]] ''enteropathy-type T-cell lymphoma'' (ETTL).
*T cell lymphoma due to [[celiac disease]].
{{Main|Enteropathy-associated T-cell lymphoma}}
 
===Microscopic===
Features:
*Epithelium preserved.
*Small lymphoid cells in the mucosa and submucosa.
 
Image: [http://commons.wikimedia.org/wiki/File:Enteropathy-associated_T_cell_lymphoma_-_low_mag.jpg EATL (WC)].


==Angioimmunoblastic T-cell lymphoma==
==Angioimmunoblastic T-cell lymphoma==
*Abbreviated ''AITL''.
===General===
*Rare.
**Common among T-cell lymphomas.
*Middle age ''or'' elderly.


===Microscopic===
===Microscopic===
Features:
Features:
*Clear cytoplasm.
*Intermediate size cells with:
**+/-[[Vesicular nuclei]].
**Clear, moderate cytoplasm.
*"Empty" sinus; subcapsular sinuses "open".
*"Empty" sinus; subcapsular sinuses "open".
Images:
*[http://www.ijpmonline.org/viewimage.asp?img=IndianJPatholMicrobiol_2010_53_4_640_72010_f4.jpg AITL (ijpmonline.org)].<ref name=pmid21045384>{{Cite journal  | last1 = Bal | first1 = M. | last2 = Gujral | first2 = S. | last3 = Gandhi | first3 = J. | last4 = Shet | first4 = T. | last5 = Epari | first5 = S. | last6 = Subramanian | first6 = PG. | title = Angioimmunoblastic T-Cell lymphoma: a critical analysis of clinical, morphologic and immunophenotypic features. | journal = Indian J Pathol Microbiol | volume = 53 | issue = 4 | pages = 640-5 | month =  | year =  | doi = 10.4103/0377-4929.72010 | PMID = 21045384 | URL = http://www.ijpmonline.org/text.asp?2010/53/4/640/72010 }}</ref>
*[http://path.upmc.edu/cases/case650.html AITL - several images (upmc.edu)]


===IHC===
===IHC===
Features - positives:<ref name=pmid21045384/>
*CD3 +ve.
*CD5 +ve.
*CD43 +ve.
Others:
*CD4 +ve and CD8 +ve with CD4>CD8.
*CD20 +ve/-ve!
*CD10 +ve/-ve!
*CD21 +ve -- prominent FDC meshworks;<ref>URL: [http://path.upmc.edu/cases/case650/dx.html http://path.upmc.edu/cases/case650/dx.html]. Accessed on: 27 January 2012.</ref> tumour cell not +ve.
Negatives:
*CD30 -ve.<ref name=pmid21045384/>
*CD15 -ve.<ref name=pmid21045384/>
*CD7 -ve.
*CD7 -ve.
*CD20 +ve.
*TIA-1 -ve.
*TIA-1 -ve.


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*Abbreviated ''PMBL''.
*Abbreviated ''PMBL''.
*[[AKA]] ''primary mediastinal large B-cell lymphoma''.
*[[AKA]] ''primary mediastinal large B-cell lymphoma''.
{{Main|Primary mediastinal B-cell lymphoma}}
==Anaplastic large cell lymphoma==
*Abbreviated ''ALCL''.
{{Main|Anaplastic large cell lymphoma}}
==Cutaneous T cell lymphoma==
:''See [[Dermatologic_neoplasms#Cutaneous_T_cell_lymphoma|Dermatologic neoplasms]]''.


===General===
===General===
Features:<ref name=pmid19074109 >{{Cite journal  | last1 = Johnson | first1 = PW. | last2 = Davies | first2 = AJ. | title = Primary mediastinal B-cell lymphoma. | journal = Hematology Am Soc Hematol Educ Program | volume =  | issue =  | pages = 349-58 | month =  | year = 2008 | doi = 10.1182/asheducation-2008.1.349 | PMID = 19074109 }}</ref><ref name=pmid20207294>{{Cite journal  | last1 = Coso | first1 = D. | last2 = Rey | first2 = J. | last3 = Bouabdallah | first3 = R. | title = [Primary mediastinal B-cell lymphoma] | journal = Rev Pneumol Clin | volume = 66 | issue = 1 | pages = 32-5 | month = Feb | year = 2010 | doi = 10.1016/j.pneumo.2009.12.007 | PMID = 20207294 }}</ref>  
*Abbreviated as ''CTCL''.
*Rare.
**''Sézary syndrome'' is a subset of CTCL.
*Young adults.
 
===Microscopic===
Features:<ref>URL: [http://emedicine.medscape.com/article/209091-overview http://emedicine.medscape.com/article/209091-overview]. Accessed on: 19 August 2010.</ref>
*Cerebriform nucleus (Sézary cell):<ref>URL: [http://emedicine.medscape.com/article/204529-diagnosis http://emedicine.medscape.com/article/204529-diagnosis]. Accessed on: 19 August 2010.</ref>
**Hyperchromatic.
**"Convoluted" = twisted, coiled.<ref>URL: [http://dictionary.reference.com/browse/convoluted http://dictionary.reference.com/browse/convoluted]. Accessed on: 19 August 2010.</ref>
 
Images:
*[http://www.flickr.com/photos/32549645@N02/3040759595/ Sézary cell (flickr.com)].
*[http://www.wadsworth.org/chemheme/heme/glass/cytopix/slide015_sezary2.jpg Sézary cell (wadsworth.org)].
 
==T-cell large granular lymphocytic leukemia==
===General===
*May be seen in the context of [[Felty syndrome]].


===Microscopic===
===Microscopic===
Features:
Features:
*Similar to DLBCL.
*Large cell lymphoma.


Images:
Images:
*[http://commons.wikimedia.org/wiki/File:Primary_mediastinal_large_B-cell_lymphoma_-_very_high_mag.jpg PMBL - very high mag. (WC)].
*[http://path.upmc.edu/cases/case695.html T-cell large granular lymphocytic leukemia (upmc.edu)].
*[http://commons.wikimedia.org/wiki/File:Primary_mediastinal_large_B-cell_lymphoma_-_intermed_mag.jpg PMBL - intermed. mag. (WC)].


==Anaplastic large cell lymphoma==
===IHC===
*CD57 +ve -- '''important'''.
*CD3 +ve.
*CD5 +ve.
*CD45 +ve.
 
==Lymphoplasmacytic lymphoma==
:''Waldenström macroglobulinemia'' redirects here.
===General===
===General===
*Abbreviated '''ALCL'''.
Features:<ref name=Ref_PCPBoD8_325>{{Ref PCPBoD8|325}}</ref>
*May look a lot like a carcinoma.
*B cell neoplasm.
**Often subcapsular in LNs.
*Secretes monoclonal IgM.
*Usually T-cell derived.
*Alk IHC:
** +ve = good prognosis.
** -ve = bad prognosis.


DDx:
Note:
*Hodgkin's lymphoma.
*''Waldenström macroglobulinemia'' is a type of lymphoplasmacytic lymphoma<ref name=pmid22507796>{{Cite journal  | last1 = Gertz | first1 = MA. | title = Waldenström macroglobulinemia. | journal = Hematology | volume = 17 Suppl 1 | issue =  | pages = 112-6 | month = Apr | year = 2012 | doi = 10.1179/102453312X13336169156212 | PMID = 22507796 }}</ref> - it is characterized by:
**''Hyperviscosity syndrome''.
**Bony destruction (seen in [[multiple myeloma]]) is absent.<ref name=Ref_PCPBoD8_325>{{Ref PCPBoD8|325}}</ref>
**Over 90% of patients have the MYD88 L265P somatic mutation.<ref name=pmid=22931316>{{cite journal |vauthors=Treon SP, Xu L, Yang G, Zhou Y, Liu X, Cao Y, Sheehy P, Manning RJ, Patterson CJ, Tripsas C, Arcaini L, Pinkus GS, Rodig SJ, Sohani AR, Harris NL, Laramie JM, Skifter DA, Lincoln SE, Hunter ZR |title=MYD88 L265P somatic mutation in Waldenström's macroglobulinemia |journal=N Engl J Med |volume=367 |issue=9 |pages=826–33 |date=August 2012 |pmid=22931316 |doi=10.1056/NEJMoa1200710 |url=}}</ref>
 
====Clinical====
Features:<ref name=Ref_PCPBoD8_325>{{Ref PCPBoD8|325}}</ref>
*Symptoms of blood hyperviscosity - these include:
**Visual impairment.
**Neurologic impairment.
**Bleeding.
**[[Cryoglobulinemia]] - may have ''Raynaud phenomenon''.
*Hemolysis.
*Bence-Jones proteinuria - seen in over half of patients.<ref name=pmid11797112>{{Cite journal  | last1 = Kyrtsonis | first1 = MC. | last2 = Vassilakopoulos | first2 = TP. | last3 = Angelopoulou | first3 = MK. | last4 = Siakantaris | first4 = P. | last5 = Kontopidou | first5 = FN. | last6 = Dimopoulou | first6 = MN. | last7 = Boussiotis | first7 = V. | last8 = Gribabis | first8 = A. | last9 = Konstantopoulos | first9 = K. | title = Waldenström's macroglobulinemia: clinical course and prognostic factors in 60 patients. Experience from a single hematology unit. | journal = Ann Hematol | volume = 80 | issue = 12 | pages = 722-7 | month = Dec | year = 2001 | doi = 10.1007/s00277-001-0385-8 | PMID = 11797112 }}</ref>
 
Treatment:
*Watchful waiting or chemotherapy.
*Hyperviscosity syndrome: plasmapheresis.


===Microscopic===
===Microscopic===
Features:
Features:<ref name=Ref_PCPBoD8_325>{{Ref PCPBoD8|325}}</ref>
*Large cells with eosinophilic cytoplasm.
*Plasmacytoid lymphocytes.  
*Usu. appear cohesive.
*Mixed inflammatory infiltrate with [[mast cell]]s, [[plasma cell]]s, lymphocytes.
*May be subcapsular.
 
*Large multinucleated cell - "wreath cell" - '''key feature'''.
DDx:
*[[Plasma cell neoplasm]].


===IHC===
===IHC===
Features:
Features:<ref name=pmid20654153>{{Cite journal  | last1 = Liu | first1 = EB. | last2 = Zhang | first2 = PH. | last3 = Li | first3 = ZQ. | last4 = Sun | first4 = Q. | last5 = Yang | first5 = QY. | last6 = Fang | first6 = LH. | last7 = Sun | first7 = FJ. | last8 = Qiu | first8 = LG. | title = [Clinicopathologic features of lymphoplasmacytic lymphoma]. | journal = Zhonghua Bing Li Xue Za Zhi | volume = 39 | issue = 5 | pages = 308-12 | month = May | year = 2010 | doi =  | PMID = 20654153 }}</ref>
*Variable CD30 +ve. (???)
*PAX5 +ve.
*CD45 +ve. (???)
*CD20 +ve.
*CD38 +ve.  
*CD138 +ve.


==Cutaneous T cell lymphoma==
Others:<ref name=pmid20654153/>
:''See [[Dermatologic_neoplasms#Cutaneous_T_cell_lymphoma|Dermatologic neoplasms]]''.
*CD5 -ve.
*CD10 -ve.
*CD23 -ve.
*CyclinD1 -ve.
*CD3 -ve.
*CD7 -ve.


==Adult T-cell leukemia/lymphoma==
===General===
===General===
*Abbreviated as ''CTCL''.
*Etiology: ''Human T-cell Lymphoma Virus 1 (HTLV-1).<ref name=Ref_PCPBoD8_327>{{Ref PCPBoD8|327}}</ref>
**''Sézary syndrome'' is a subset of CTCL.
*Poor prognosis ~ 1 year survival with treatment.


===Microscopic===
===Microscopic===
Features:<ref>URL: [http://emedicine.medscape.com/article/209091-overview http://emedicine.medscape.com/article/209091-overview]. Accessed on: 19 August 2010.</ref>
Features:<ref name=Ref_PCPBoD8_328>{{Ref PCPBoD8|328}}</ref>
*Cerebriform nucleus (Sézary cell):<ref>URL: [http://emedicine.medscape.com/article/204529-diagnosis http://emedicine.medscape.com/article/204529-diagnosis]. Accessed on: 19 August 2010.</ref>
*Cloverleaf nuclei.
**Hyperchromatic.
**Nuclei with multiple lobulations.
**"Convoluted" = twisted, coiled.<ref>URL: [http://dictionary.reference.com/browse/convoluted http://dictionary.reference.com/browse/convoluted]. Accessed on: 19 August 2010.</ref>
 
Image:
*[http://www.pathpedia.com/education/eatlas/histopathology/blood_cells/adult_t-cell_leukemia_htlv1-positive_acute_form/adult-t-cell-leukemia-htlv1-%5B2-bl095-2%5D.jpeg?Width=600&Height=450&Format=4 Cloverleaf nucleus (pathpedia.com)].<ref>URL: [http://www.pathpedia.com/education/eatlas/histopathology/blood_cells/adult_t-cell_leukemia_htlv1-positive_acute_form.aspx http://www.pathpedia.com/education/eatlas/histopathology/blood_cells/adult_t-cell_leukemia_htlv1-positive_acute_form.aspx]. Accessed on: 7 February 2012.</ref>
 
===IHC===
Features:<ref name=pmid19165640>{{Cite journal  | last1 = Bittencourt | first1 = AL. | last2 = Barbosa | first2 = HS. | last3 = Vieira | first3 = MD. | last4 = Farré | first4 = L. | title = Adult T-cell leukemia/lymphoma (ATL) presenting in the skin: clinical, histological and immunohistochemical features of 52 cases. | journal = Acta Oncol | volume = 48 | issue = 4 | pages = 598-604 | month =  | year = 2009 | doi = 10.1080/02841860802657235 | PMID = 19165640 |URL = http://informahealthcare.com/doi/pdf/10.1080/02841860802657235 }}</ref>
*CD3 +ve.
*CD5 +ve.
*CD25 +ve.
*CD45 +ve.
*HTLV-1 +ve.
 
Others:<ref name=pmid19165640/>
*CD7 -ve.
*CD20 -ve.
*CD79a -ve.
 
==Hepatosplenic T-cell lymphoma==
{{Main|Hepatosplenic T-cell lymphoma}}


Images:
==Extranodal NK/T-cell lymphoma, nasal type==
*[http://www.flickr.com/photos/32549645@N02/3040759595/ Sézary cell (flickr.com)].
*Abbreviated as ''ENKL'' or ''ENKTCL''.
*[http://www.wadsworth.org/chemheme/heme/glass/cytopix/slide015_sezary2.jpg Sézary cell (wadsworth.org)].
*[[AKA]] ''extranodal natural kill lymphoma''
*[[AKA]] ''angiocentric lymphoma''.
{{Main|Extranodal NK/T-cell lymphoma, nasal type}}


==Table of lymphoma==
==Table of lymphoma==
Line 344: Line 489:
! Histomorphology
! Histomorphology
! IHC
! IHC
! Translocations
! [[Translocations]]
! Clinical
! Clinical
! Prevalence
! Prevalence
! DDx
! DDx
|-
|-
| Follicular lymphoma
| [[Follicular lymphoma]]
| small (centrocytes)
| small (centrocytes)
| lymph node, germinal center
| lymph node, germinal center
Line 359: Line 504:
| DLBCL, other small cell lymphomas
| DLBCL, other small cell lymphomas
|-
|-
| Mantle cell lymphoma
| [[Mantle cell lymphoma]]
| small, monomorphic
| small, monomorphic
| lymph node, mantle zone
| lymph node, mantle zone
Line 367: Line 512:
| indolent ???
| indolent ???
| uncommon
| uncommon
| other small cell lymphomas, PTGC, Castleman disease, Burkitt's lymphoma
| other [[small cell lymphomas]], [[PTGC]], [[Castleman disease]], [[Burkitt's lymphoma]]
|-
|-
| Extranodal marginal zone lymphoma (MALT lymphoma)
| Extranodal marginal zone lymphoma (MALT lymphoma)
Line 381: Line 526:
| Precursor B-cell lymphoblastic lymphoma/ leukemia
| Precursor B-cell lymphoblastic lymphoma/ leukemia
| small
| small
| lymph node ???
| lymph node ???, bone marrow
| ???
| nuclei slightly large than resting lymphocytes, scant basophilic cytoplasm, irregular nuclear membrane, no nucleoli, stippled chromatin<ref name=Ref_PCPBoD8_315>{{Ref PCPBoD8|315}}</ref>
| CD10+, CD5-, TdT+, CD99+<ref name=Ref_Lester95>{{Ref Lester|95}}</ref>
| CD10+, CD5-, TdT+, CD99+<ref name=Ref_Lester95>{{Ref Lester|95}}</ref>
| translocations ???
| +/-t(12;21)<ref name=Ref_PCPBoD8_317>{{Ref PCPBoD8|317}}</ref>
| aggressive ???
| aggressive ???
| uncommon
| uncommon
| small cell lymphomas
| [[small cell lymphomas]]
|-  
|-  
| Hairy cell lymphoma
| [[Hairy cell leukemia]]
| small
| small
| bone marrow, peripheral blood
| bone marrow, peripheral blood
Line 397: Line 542:
| splenomegaly, no lymphadenopathy, pancytopenia, good prognosis with Tx
| splenomegaly, no lymphadenopathy, pancytopenia, good prognosis with Tx
| uncommon
| uncommon
| small cell lymphomas  
| small cell leukemias/lymphomas (e.g. [[SMZL]])
|-
|-
| Burkitt's lymphoma
| [[Burkitt's lymphoma]]
| medium, monomorphic
| medium, monomorphic
| lymph node, germinal center (???)
| lymph node, germinal center (???)
Line 405: Line 550:
| CD10+, BCL6+, BCL2-
| CD10+, BCL6+, BCL2-
| t(8;14) (q24;q32)  
| t(8;14) (q24;q32)  
| rapid growth, may be associated with EBV, HIV/AIDS
| rapid growth, may be associated with [[EBV]], [[HIV]]/AIDS
| uncommon
| uncommon
| DLBCL, mantle cell lymphoma
| DLBCL, mantle cell lymphoma
|-
|-
| Diffuse large B cell lymphoma
| [[Diffuse large B cell lymphoma]]
| large cells (>2x RBC, often larger), variable size
| large cells (>2x RBC, often larger), variable size
| lymph node usually, germinal center
| lymph node usually, germinal center
| sheets of large discohesive cells; if only nodular = follicular lymphoma
| sheets of large discohesive cells; if only nodular = follicular lymphoma
| MIB-1 >40%
| MIB1 >40%
| none / like follicular lymphoma t(14,18) / c-MYC (like Burkitt lymphoma)
| none / like follicular lymphoma t(14,18) / c-MYC (like Burkitt lymphoma)
| poor prognosis
| poor prognosis
| very common
| very common
| Burkitt lymphoma, ALCL, Hodgkin lymphoma
| Burkitt lymphoma, [[ALCL]], [[Hodgkin lymphoma]]
|-  
|-  
| Primary mediastinal B-cell lymphoma
| [[Primary mediastinal B-cell lymphoma]]
| large (>2x RBC, often larger), variable size
| large (>2x RBC, often larger), variable size
| mediastinum  
| mediastinum  
Line 425: Line 570:
| IHC ?
| IHC ?
| translocations ?
| translocations ?
| predominantly young adults, better prognosis than DLBCL
| predominantly young adults, better prognosis than [[DLBCL]]
| uncommon
| uncommon
| DLBCL  
| DLBCL  
|-
| B cell small lymphocytic lymphoma /<br>chronic lymphocytic leukemia
| small
| lymph node (???)
| proliferation centres
| CD5+, CD23+, CD43+, cyclin D1-
| trisomy 12; deletions of 11q, 13q, 17p<ref>{{Ref PCPBoD8|318}}</ref>
| good prognosis / indolent course
| common
| other [[small cell lymphomas]]
|- <!--
|- <!--
| Name
| Name
Line 452: Line 607:
! DDx
! DDx
|-
|-
| Angioimmunoblastic lymphoma
| [[Angioimmunoblastic lymphoma]]
| size of cells ?
| size of cells ?
| site ?
| site ?
Line 462: Line 617:
| DDx ?  
| DDx ?  
|-
|-
| Enteropathy-type T cell lymphoma
| [[Enteropathy-type T cell lymphoma]]
| size of cells ?
| size of cells ?
| site ?
| site ?
Line 482: Line 637:
| DDx ?  
| DDx ?  
|-
|-
| Adult T-cell lymphoma / leukemia
| [[Adult T-cell leukemia/lymphoma|Adult T-cell lymphoma / leukemia]]
| size of cells ?
| size of cells ?
| site ?
| site ?
| histomorphology ?
| cloverleaf nuclei (multilobular nuclei)
| IHC ?
| IHC ?
| translocations ?
| translocations ?
| clinical ?
| poor prognosis ~ 1 year survival w/ Tx; d/t HTLV-1
| prevalence ?
| prevalence ?
| DDx ?  
| DDx ?  
Line 502: Line 657:
| DDx ?  
| DDx ?  
|-
|-
| Anaplastic large cell lymphoma
| [[Anaplastic large cell lymphoma]]
| size of cells ?
| large
| site ?
| deep & subcapsular sinuses of [[LN]]
| histomorphology ?
| eosinophilic cytoplasm, nucleoli, often cohesive, wreath cell (C-shaped nucleus)
| CD30+/-, Alk+/-
| CD30+/-, Alk+/-, CD4+, CD3-
| translocations ?
| t(2,5)(p23;q35)<ref name=pmid8547653>{{cite journal |author=Lamant L, Meggetto F, al Saati T, ''et al.'' |title=High incidence of the t(2;5)(p23;q35) translocation in anaplastic large cell lymphoma and its lack of detection in Hodgkin's disease. Comparison of cytogenetic analysis, reverse transcriptase-polymerase chain reaction, and P-80 immunostaining |journal=Blood |volume=87 |issue=1 |pages=284–91 |year=1996 |month=January |pmid=8547653 |doi= |url=}}</ref>
| clinical ?
| clinical ?
| prevalence ?
| uncommon
| DDx ?
| carcinoma
|-
|-
| Extranodal NK / T cell lymphoma nasal type
| [[Extranodal NK/T-cell lymphoma, nasal type|Extranodal NK/T cell lymphoma nasal type]]
| size of cells ?
| large ???
| site ?
| nasal ???
| histomorphology ?
| histomorphology ?
| EBER+, CD16+, CD56+, CD57-, TIA-1+, Granzyme B+
| EBER+, CD16+, CD56+, CD57-, TIA-1+, Granzyme B+
| translocations ?
| translocations ?
| clinical ?
| common in East Asia
| prevalence ?
| uncommon
| DDx ?  
| DDx ?  
|-
|-