Difference between revisions of "Lymphoma"

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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.   


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A general introduction to haematopathology is in the ''[[haematopathology]]'' article.
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).
**Not pathognomonic.
*Reed-Sternberg cells = [[Hodgkin's lymphoma]].
*Reed-Sternberg cells = [[Hodgkin's lymphoma]].
**Large cell - very large nucleus.
**Large cell - very large nucleus.
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Others:
Others:
*AE1/AE3 -- to r/o carcinoma.
*[[AE1/AE3]] -ve -- to r/o carcinoma.


====T cell markers====
====T cell markers====
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*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.
*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>.
*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.
*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====
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*TdT.
*TdT.
*CD34.
*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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==Intravascular lymphoma==
==Intravascular lymphoma==
*[[AKA]] ''angiotropic lymphoma'', ''intravascular malignant lymphomatosis'', ''malignant angioendotheliomatosis''
*Rare.
*Rare.
*Usually B-cell lineage (see below ''intravascular large B-cell lymphoma'').
*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>
**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>
===Intravascular large B-cell lymphoma===
====General====
*[[AKA]] ''angiotropic lymphoma'', ''intravascular malignant lymphomatosis'', ''malignant angioendotheliomatosis''.
=====Clinical=====
*Often a non-specific presentation.<ref name=pmid11579120>{{cite journal |author=Lapkuviene O, Forchetti D, Roepke JE |title=Unusual sites of involvement by hematologic malignancies. Case 1. Intravascular large B-cell lymphoma presenting with CNS symptoms |journal=J. Clin. Oncol. |volume=19 |issue=19 |pages=3988–91 |year=2001 |month=October |pmid=11579120 |doi= |url=http://jco.ascopubs.org/content/19/19/3988.full}}</ref>
**+/-Fever.
**+/-Multiple infarcts.
**+/-Non-specific skin lesions.
====Microscopic====
Features:
*Abundant atypical intravascular lymphoid cells that are:
**Large (~2-3X size of a mature lymphocyte or RBC).
**Nucleolus prominent.
Notes:
*It may be hard to find RBCs in the vessels.
*Looks sorta like a [[DLBCL]] -- but is intravascular.
Images:
*[http://en.wikipedia.org/wiki/File:Intravascular_lymphoma_-_high_mag.jpg Intravascular lymphoma - high mag. (WC)].
*[http://www.pathconsultddx.com/pathCon/largeImage?pii=S1559-8675%2806%2970476-4&figureId=fig5 CNS lymphoma (pathconsultddx.com)].<ref>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970476-4 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970476-4]. Accessed on: 27 October 2010.</ref>
*[http://jco.ascopubs.org/content/19/19/3988/F1.expansion.html ILBCL (ascopubs.org)].<ref name=pmid11579120/>
*[http://theoncologist.alphamedpress.org/cgi/content/full/11/8/923/F1 ILBCL in bone marrow (alphamedpress.org)].
*[http://annonc.oxfordjournals.org/content/13/9/1503/F1.expansion.html ILBCL - various images (oxfordjournals.org)].
====IHC====
Features:<ref name=pmid11579120/>
*CD20 +ve - '''key feature'''.
*CD3 -ve.
*CD34 -ve.


==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 myeloma''.
*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.
 
===IHC===
*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.
**Also +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.
*CD38. (???)
 
===Molecular===
*t(4;14)(p16.3;q32.3) / IGH–MMSET.<ref name=pmid9787135>{{Cite journal  | last1 = Chesi | first1 = M. | last2 = Nardini | first2 = E. | last3 = Lim | first3 = RS. | last4 = Smith | first4 = KD. | last5 = Kuehl | first5 = WM. | last6 = Bergsagel | first6 = PL. | title = The t(4;14) translocation in myeloma dysregulates both FGFR3 and a novel gene, MMSET, resulting in IgH/MMSET hybrid transcripts. | journal = Blood | volume = 92 | issue = 9 | pages = 3025-34 | month = Nov | year = 1998 | doi =  | PMID = 9787135 }}</ref>
**Assoc. with poor prognosis.<ref name=pmid12393535>{{Cite journal  | last1 = Keats | first1 = JJ. | last2 = Reiman | first2 = T. | last3 = Maxwell | first3 = CA. | last4 = Taylor | first4 = BJ. | last5 = Larratt | first5 = LM. | last6 = Mant | first6 = MJ. | last7 = Belch | first7 = AR. | last8 = Pilarski | first8 = LM. | title = In multiple myeloma, t(4;14)(p16;q32) is an adverse prognostic factor irrespective of FGFR3 expression. | journal = Blood | volume = 101 | issue = 4 | pages = 1520-9 | month = Feb | year = 2003 | doi = 10.1182/blood-2002-06-1675 | PMID = 12393535 }}</ref>


==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 sprue]].
{{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===
*Abbreviated ''ALCL''.
*CD57 +ve -- '''important'''.
*CD3 +ve.
*CD5 +ve.
*CD45 +ve.


==Lymphoplasmacytic lymphoma==
:''Waldenström macroglobulinemia'' redirects here.
===General===
===General===
*May look a lot like a carcinoma.
Features:<ref name=Ref_PCPBoD8_325>{{Ref PCPBoD8|325}}</ref>
**Often subcapsular in LNs.
*B cell neoplasm.
*Usually T-cell derived.
*Secretes monoclonal IgM.
*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>
 
====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.  
*Usually appear cohesive.
*Mixed inflammatory infiltrate with [[mast cell]]s, [[plasma cell]]s, lymphocytes.
*May be subcapsular in a [[lymph node]] and mimic a carcinoma.
 
*''Hallmark cells'' = "horseshoe-shaped or donut-shaped nucleus + eosinophilic paranuclear region"<ref name=pmid17941004>{{Cite journal  | last1 = Rapkiewicz | first1 = A. | last2 = Wen | first2 = H. | last3 = Sen | first3 = F. | last4 = Das | first4 = K. | title = Cytomorphologic examination of anaplastic large cell lymphoma by fine-needle aspiration cytology. | journal = Cancer | volume = 111 | issue = 6 | pages = 499-507 | month = Dec | year = 2007 | doi = 10.1002/cncr.23120 | PMID = 17941004 | url=http://onlinelibrary.wiley.com/doi/10.1002/cncr.23120/full}}</ref><ref name=pmid12419758>{{Cite journal  | last1 = Ponzoni | first1 = M. | last2 = Terreni | first2 = MR. | last3 = Ciceri | first3 = F. | last4 = Ferreri | first4 = AJ. | last5 = Gerevini | first5 = S. | last6 = Anzalone | first6 = N. | last7 = Valle | first7 = M. | last8 = Pizzolito | first8 = S. | last9 = Arrigoni | first9 = G. | title = Primary brain CD30+ ALK1+ anaplastic large cell lymphoma ('ALKoma'): the first case with a combination of 'not common' variants. | journal = Ann Oncol | volume = 13 | issue = 11 | pages = 1827-32 | month = Nov | year = 2002 | doi =  | PMID = 12419758 }}</ref> - '''key feature'''.
DDx:
**The donut-shaped version is also known as a "wreath cell"<ref>{{Cite journal  | last1 = Amin | first1 = HM. | last2 = Lai | first2 = R. | title = Pathobiology of ALK+ anaplastic large-cell lymphoma. | journal = Blood | volume = 110 | issue = 7 | pages = 2259-67 | month = Oct | year = 2007 | doi = 10.1182/blood-2007-04-060715 | PMID = 17519389 | url=http://bloodjournal.hematologylibrary.org/content/110/7/2259.full.html}}</ref> - large (multi-nucleated) cells with (morphologically) one toroidal-shaped nucleus.
*[[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>
*CD30 +ve.  
*PAX5 +ve.
*ALK-1 -ve/+ve; strongly supports ALCL Dx if +ve.
*CD20 +ve.  
*CD45 +ve.
*CD38 +ve.  
*CD4 +ve.
*CD138 +ve.
*CD3 -ve/+ve.
*CD7 -ve/+ve.
*EMA +ve.


===Molecular===
Others:<ref name=pmid20654153/>
*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> - can be detected with [[FISH]] break apart probe.
*CD5 -ve.
 
*CD10 -ve.
==Cutaneous T cell lymphoma==
*CD23 -ve.
:''See [[Dermatologic_neoplasms#Cutaneous_T_cell_lymphoma|Dermatologic neoplasms]]''.
*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 453: Line 493:
! DDx
! DDx
|-
|-
| Follicular lymphoma
| [[Follicular lymphoma]]
| small (centrocytes)
| small (centrocytes)
| lymph node, germinal center
| lymph node, germinal center
Line 463: Line 503:
| 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 471: Line 511:
| 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 485: Line 525:
| 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 501: Line 541:
| 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 513: Line 553:
| 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 529: Line 569:
| 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 556: Line 606:
! DDx
! DDx
|-
|-
| Angioimmunoblastic lymphoma
| [[Angioimmunoblastic lymphoma]]
| size of cells ?
| size of cells ?
| site ?
| site ?
Line 566: Line 616:
| DDx ?  
| DDx ?  
|-
|-
| Enteropathy-type T cell lymphoma
| [[Enteropathy-type T cell lymphoma]]
| size of cells ?
| size of cells ?
| site ?
| site ?
Line 586: Line 636:
| 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 606: Line 656:
| DDx ?  
| DDx ?  
|-
|-
| Anaplastic large cell lymphoma
| [[Anaplastic large cell lymphoma]]
| large
| large
| deep & subcapsular sinuses of LN
| deep & subcapsular sinuses of [[LN]]
| eosinophilic cytoplasm, nucleoli, often cohesive, wreath cell (C-shaped nucleus)
| eosinophilic cytoplasm, nucleoli, often cohesive, wreath cell (C-shaped nucleus)
| CD30+/-, Alk+/-, CD4+, CD3-
| CD30+/-, Alk+/-, CD4+, CD3-
Line 616: Line 666:
| carcinoma  
| 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 ?  
|-
|-
48,453

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