Libre Pathology - User contributions [en]

Libre Pathology news:New offline version of Libre Pathology

2025-09-17T10:38:43Z

Maxwell: /* Acknowledgements */

[[Image:Libre Pathology - logo - white background.png|200px|thumb|The [[Libre Pathology]] logo.]]
A '''new offline version of Libre Pathology''' is available at ''kiwix.org'' and will be updated quarterly by the team at Kiwix.

It has been a long term goal to give ''you'' the whole site. After all, ''libre'' (free), is in the name ''[[Libre Pathology]]''.

We hope the offline version helps make pathology information available to those that do not have an internet connection ''or'' a less than optimal one.

==User experience==
Kiwix has a strong built-in search feature, and navigation otherwise is similar to online. The hyperlinks work.

Unfortunately, the [[Quizzes|short spot diagnosis quizzes]] and [[Cases|learning with simulated cases]] do not work.
Images are lower resolution to make a file that is approximately 80 MB.

==Setting up an offline version of Libre Pathology==
# Install Kiwix - takes less than 5 minutes.
#* Android: https://play.google.com/store/apps/details?id=org.kiwix.kiwixmobile&hl=en
#* MacOS: https://itunes.apple.com/us/app/kiwix/id997079563
#* Windows: https://download.kiwix.org/release/kiwix-tools/kiwix-tools_win-i686.zip
#* Linux (64 bit): https://download.kiwix.org/release/kiwix-tools/kiwix-tools_linux-x86_64.tar.gz
# Download the Libre Pathology ZIM file.
#* A direct link is here: https://download.kiwix.org/zim/other/librepathology_en_all_maxi_2025-09.zim
# Open Kiwix and load the ZIM file - this should takes less than 2 minutes.
# Enjoy Libre Pathology offline!

==Acknowledgements==
Special thanks go to [[User:Maxwell|Maxwell Martin]] (MS4 at Sidney Kimmel Medical College) for his work on generating a new ZIM file and coordinating with Kiwix.
[[Libre Pathology news:Offline version of Libre Pathology|An earlier offline version]] was inspired by Dr. Parkash.

Emmanuel Engelhart made this all possible. He did all the heavy lifting in this project; he developed Kiwix, initially that [[Wikipedia]] could go offline.

==See also==
*[[Libre Pathology news]].

==External links==
*[https://meta.wikimedia.org/wiki/Kiwix_-_Wikipedia_Offline Wikipedia Offline (wikimedia.org)].
*[https://en.wikipedia.org/wiki/Kiwix Kiwix (wikipedia.org)].

[[Category:Libre Pathology news]]

Lymphoma

2025-07-13T21:07:55Z

Maxwell: /* Lymphoplasmacytic lymphoma */

[[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.

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

Other categorizations:
*T cell lymphomas (rare).
*B cell lymphomas (more common).

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

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

===Histologic classification===
#"Size".
#Nodularity.

===="Size"====
*The single most important factor for classifying lymphomas.
*Not really based on size.

{| class="wikitable"
|
| "Large"
| "Small"
| Utility
|-
| Nucleoli
| present
| absent
| most discriminative
|-
| Size
| >2x RBC dia.
| <2x RBC dia.
| moderate
|-
| Chromatin pattern
| "open" (pale)
| "closed"
| moderate/minimal
|-
| Cytoplasm
| mold-minimal <br>basophilic cytoplasm
| scant cytoplasm
| minimal
|}

====Histologic terms====
*Lymphomas = cells look discohesive, may be difficult to differentiate from poor differentiated carcinoma.
*[[Auer rods]] = [[acute myeloid leukemia]].
**Granular cytoplasmic rod (0.5-1 x4-6 micrometres).
**Not pathognomonic.
*Reed-Sternberg cells = [[Hodgkin's lymphoma]].
**Large cell - very large nucleus.
***Classically binucleated.
*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>
***''Mott cell'' is a cell that contains Russell bodies.<ref name=pmid3979421/>
*Effacement of nodal architecture.
*Loss of proliferation centers.

===IHC===
====General====
*CD45.
**AKA ''common lymphocyte antigen''.
**Useful to differentiate from carcinomas (e.g. small cell carcinoma).

Others:
*[[AE1/AE3]] -ve -- to r/o carcinoma.

====T cell markers====
*CD2 -- T cell marker (all T cells).
*CD3 -- T cell marker (all T cells).
**CD4 -- subset of T cells.
**CD8 -- subset of T cells.
*CD7 -- often lost first in T cell lymphomas.
*CD5 -- +ve in CLL & mantle cell lymphoma.
*CD43 -- +ve in mantle cell lymphoma

*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 - 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.
====B cell markers====
*CD20 -- B cell marker.
**CD19 -- B cell marker - used for [[flow cytometry]].
*PAX5 -- nuclear staining.<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/167414 http://www.ncbi.nlm.nih.gov/omim/167414]. Accessed on: 18 August 2010.</ref>
**Image: [http://www.pathologypics.com/PictView.aspx?ID=529 PAX5 in Hodgkin's lymphoma].
*CD79a.

*CD10 -- follicle center.
*BCL6 -- follicle center.
*BCL2 -- normally negative in germinal centers (GCs), marks T cell; +ve in small B cell lymphomas.
*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====
*CD23 -- 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====
=====Classic=====
*CD30 +ve -- Hodgkin's lymphoma (most sensitive).
*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==
{{main|Hodgkin's lymphoma}}
===General===
*Abbreviated ''HL''.

===Microscopic===
By definition, HL has Reed-Sternberg cells (RSCs).

====Classical HL====
Features (classic HL):
*Reed-Sternberg cell.
**Large binucleated cell.
***May be multinucleated.
***May have a horseshoe-like shape.
**Macronucleolus - approximately the size of a RBC (~8 micrometers).
**Well-defined cell border.

Notes:
*Large mononuclear cells are common (so called "mononuclear RSCs") but not diagnostic.

Images (classic HL):
*[http://commons.wikimedia.org/wiki/File:Hodgkin_lymphoma_cytology_large.jpg HL mixed cellularity - cytology (WC)].
*[http://commons.wikimedia.org/wiki/File:Hodgkin_lymphoma_cytology_small.jpg HL mixed cellularity - cytology (WC)].
*[http://en.wikipedia.org/wiki/File:Hodgkin_lymphoma_%281%29_mixed_cellulary_type.jpg HL mixed cellularity (WC)].

=====Subtypes=====
There are four CHL subtypes:<ref name=Ref_WMSP567>{{Ref WMSP|567}}</ref>
#Nodular sclerosis CHL - ~70% of CHL.
#*Mixed cellular background - T cell, plasma cells, eosinophils, neutrophils and histiocytes.
#*Nodular sclerosing fibrosis - thick strands fibrosis.
#Mixed cellularity CHL - ~20-25% of CHL.
#*Like nodular sclerosis - but no fibrosis.
#*May be associated with HIV infection.<ref name=pmid20138008>{{cite journal |author=Sissolak G, Sissolak D, Jacobs P |title=Human immunodeficiency and Hodgkin lymphoma |journal=Transfus. Apher. Sci. |volume=42 |issue=2 |pages=131–9 |year=2010 |month=April |pmid=20138008 |doi=10.1016/j.transci.2010.01.008 |url=}}</ref>
#Lymphocyte-rich CHL - rare.
#*T lymphocytes only (no mix of cells).
#Lymphocyte-depleted CHL - rare.
#*May be associated with [[HIV]] infection.<ref name=pmid20138008/>

Memory device:
*The subtypes prevalence is in reverse alphabetical order.

====Nodular lymphocyte-predominant HL====
Features (nodular lymphocyte-predominant Hodgkin's lymphoma):
*''Popcorn cell'' (previously known as ''Lymphocytic & histiocytic'' cell (L&H cell)<ref name=pmid9499174>{{cite journal |author=Küppers R, Rajewsky K, Braeuninger A, Hansmann ML |title=L&H cells in lymphocyte-predominant Hodgkin's disease |journal=N. Engl. J. Med. |volume=338 |issue=11 |pages=763–4; author reply 764–5 |year=1998 |month=March |pmid=9499174 |doi=10.1056/NEJM199803123381113 |url=}}</ref>) - variant of RSC:
**Cells (relatively) small (compared to classic RSCs).
**Lobulated nucleus - '''key feature'''.
**Small nucleoli.
*Subtle nodularity at low power (2.5x or 5x objective).

Images (NLPHL):
*[http://webpathology.com/image.asp?case=388&n=16 Popcorn cell (webpathology.com)].
*[http://commons.wikimedia.org/wiki/File:Popcorn_cell_in_nodular_lymphocyte_predominant_Hodgkin_lymphoma_-_very_high_mag_cropped.jpg Popcorn cell (WC)].

==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==
{{main|Diffuse large B cell lymphoma}}
===General===
*Abbreviated ''DLBCL''.

===Microscopic===
Features:<ref name=Ref_PBoD676>{{Ref PBoD|676 (???)}}</ref>
*Large cells -- 4-5 times the diameter of a small lymphocytes.
*Typically have marked cell-to-cell variation in size and shape.
*Cytoplasm usu. basophilic and moderate in abundance.
*+/-Prominent nucleoli, may be peripheral and/or multiple.

Notes:
*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==
{{main|Burkitt lymphoma}}
===General===
*Abbreviated ''BL''.
*Subtyped by etiology.

===Microscopic===
Features:
*"Starry-sky pattern":
**The ''stars'' in the pattern are: tingible-body macrophages.
***''Tingible-body macrophages'' = macrophages containing apoptotic tumour cells.
**The tumour cells are the sky.
*Tumour cells:<ref name=pmid12610094>{{cite journal |author=Bellan C, Lazzi S, De Falco G, Nyongo A, Giordano A, Leoncini L |title=Burkitt's lymphoma: new insights into molecular pathogenesis |journal=J. Clin. Pathol. |volume=56 |issue=3 |pages=188–92 |year=2003 |month=March |pmid=12610094 |pmc=1769902 |doi= |url=http://jcp.bmj.com/cgi/pmidlookup?view=long&pmid=12610094}}</ref>
**Medium-sized (~1.5-2x the size of a RBC) with uniform size ("monotonous") -- '''key feature'''.
**Round nucleus.
**Small nucleoli.
**Relatively abundant cytoplasm.
**Brisk mitotic rate.

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

==Plasma cell neoplasms==
{{Main|Plasma cell neoplasms}}
*These arise from ''plasma cells''.
*[[AKA]] ''plasma cell myeloma'', ''plasmacytoma''.
*''Multiple myeloma'' (a clinical diagnosis) fits into this category.

==Acute myeloid leukemia==
===General===
*May afflicits young adult.
*Males>females.

===Complications===
*[[Chloroma]] - soft tissue mass.
*Leukostasis.
**Occurs - lungs and brain.<ref>AML. Harrison's 16th Ed.</ref>
*Hyperviscosity syndrome.
*Spontaneous bleeding with low platelet counts.

===Classification===
There are two classifications:
#FAB (French-American-British) - based on histologic appearance/maturation.
#WHO classification.

===Histology===
*Auer rods - not required to diagnose.<ref>AG. 8 July, 2009.</ref>
**Cytoplasmic granular rods in blast cells.
***Dimensions: approx. 0.5-1 x 4-6 micrometres.
**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==
*Abbreviated ''EATL''.
*[[AKA]] ''enteropathy-type T-cell lymphoma'' (ETTL).
{{Main|Enteropathy-associated T-cell lymphoma}}

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

===Microscopic===
Features:
*Intermediate size cells with:
**+/-[[Vesicular nuclei]].
**Clear, moderate cytoplasm.
*"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===
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.
*TIA-1 -ve.

==Primary mediastinal B-cell lymphoma==
*Abbreviated ''PMBL''.
*[[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===
*Abbreviated as ''CTCL''.
**''Sézary syndrome'' is a subset of CTCL.

===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===
Features:
*Large cell lymphoma.

Images:
*[http://path.upmc.edu/cases/case695.html T-cell large granular lymphocytic leukemia (upmc.edu)].

===IHC===
*CD57 +ve -- '''important'''.
*CD3 +ve.
*CD5 +ve.
*CD45 +ve.

==Lymphoplasmacytic lymphoma==
:''Waldenström macroglobulinemia'' redirects here.
===General===
Features:<ref name=Ref_PCPBoD8_325>{{Ref PCPBoD8|325}}</ref>
*B cell neoplasm.
*Secretes monoclonal IgM.

Note:
*''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===
Features:<ref name=Ref_PCPBoD8_325>{{Ref PCPBoD8|325}}</ref>
*Plasmacytoid lymphocytes.
*Mixed inflammatory infiltrate with [[mast cell]]s, [[plasma cell]]s, lymphocytes.

DDx:
*[[Plasma cell neoplasm]].

===IHC===
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>
*PAX5 +ve.
*CD20 +ve.
*CD38 +ve.
*CD138 +ve.

Others:<ref name=pmid20654153/>
*CD5 -ve.
*CD10 -ve.
*CD23 -ve.
*CyclinD1 -ve.
*CD3 -ve.
*CD7 -ve.

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

===Microscopic===
Features:<ref name=Ref_PCPBoD8_328>{{Ref PCPBoD8|328}}</ref>
*Cloverleaf nuclei.
**Nuclei with multiple lobulations.

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}}

==Extranodal NK/T-cell lymphoma, nasal type==
*Abbreviated as ''ENKL'' or ''ENKTCL''.
*[[AKA]] ''extranodal natural kill lymphoma''
*[[AKA]] ''angiocentric lymphoma''.
{{Main|Extranodal NK/T-cell lymphoma, nasal type}}

==Table of lymphoma==
===B cell lymphomas===
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
! Name
! Size of cells
! Site
! Histomorphology
! IHC
! [[Translocations]]
! Clinical
! Prevalence
! DDx
|-
| [[Follicular lymphoma]]
| small (centrocytes)
| lymph node, germinal center
| abundant atypical follicles (lack GC polarity, lack polar mantle zone), effaced sinuses, scattered large cells (centroblasts)
| CD10+, bcl-6+<ref name=Ref_Lester95>{{Ref Lester|95}}</ref>
| t(14,18)
| usually indolent, may transform to DLBCL
| very common
| DLBCL, other small cell lymphomas
|-
| [[Mantle cell lymphoma]]
| small, monomorphic
| lymph node, mantle zone
| monomorphic lymphoid, abundant mitoses. +/-scattered epithelioid histiocytes, sclerosed blood vessels
| CD5+, CD23-, CD43+, cyclin D1+<ref name=Ref_Lester95>{{Ref Lester|95}}</ref>
| t(11;14)(q13;q32)<ref>URL: [http://atlasgeneticsoncology.org/Anomalies/t1114ID2021.html http://atlasgeneticsoncology.org/Anomalies/t1114ID2021.html]. Accessed on: 10 August 2010.</ref>
| indolent ???
| uncommon
| other [[small cell lymphomas]], [[PTGC]], [[Castleman disease]], [[Burkitt's lymphoma]]
|-
| Extranodal marginal zone lymphoma (MALT lymphoma)
| small
| mucosa-associated lymphoid tissue, GI tract + elsewhere
| +/- lymphepithelial lesion (cluster of 3+ cells in epithelium)
| CD21+, CD11c+, CD5-, CD23-<ref name=Ref_Lester95>{{Ref Lester|95}}</ref>
| ???
| indolent ???
| common
| other small cell lymphomas, neuroendocrine tumours
|-
| Precursor B-cell lymphoblastic lymphoma/ leukemia
| small
| 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>
| +/-t(12;21)<ref name=Ref_PCPBoD8_317>{{Ref PCPBoD8|317}}</ref>
| aggressive ???
| uncommon
| [[small cell lymphomas]]
|-
| [[Hairy cell leukemia]]
| small
| bone marrow, peripheral blood
| perinuclear clearing, clear cytoplasm, central nucleus
| CD25+, CD103+, CD5-<ref>URL: [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022&rendertype=table&id=A34029 http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022&rendertype=table&id=A34029]. Accessed on: 20 August 2010.</ref>
| translocations ?
| splenomegaly, no lymphadenopathy, pancytopenia, good prognosis with Tx
| uncommon
| small cell leukemias/lymphomas (e.g. [[SMZL]])
|-
| [[Burkitt's lymphoma]]
| medium, monomorphic
| lymph node, germinal center (???)
| tingible-body macrophages ("starry sky" appearance), round nucleus, small nucleoli, mitoses +++
| CD10+, BCL6+, BCL2-
| t(8;14) (q24;q32)
| rapid growth, may be associated with [[EBV]], [[HIV]]/AIDS
| uncommon
| DLBCL, mantle cell lymphoma
|-
| [[Diffuse large B cell lymphoma]]
| large cells (>2x RBC, often larger), variable size
| lymph node usually, germinal center
| sheets of large discohesive cells; if only nodular = follicular lymphoma
| MIB1 >40%
| none / like follicular lymphoma t(14,18) / c-MYC (like Burkitt lymphoma)
| poor prognosis
| very common
| Burkitt lymphoma, [[ALCL]], [[Hodgkin lymphoma]]
|-
| [[Primary mediastinal B-cell lymphoma]]
| large (>2x RBC, often larger), variable size
| mediastinum
| histomorphology ?
| IHC ?
| translocations ?
| predominantly young adults, better prognosis than [[DLBCL]]
| uncommon
| 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]]
|- 
|}

===T cell lymphomas===
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
! Name
! Size of cells
! Site
! Histomorphology
! IHC
! Translocations
! Clinical
! Prevalence
! DDx
|-
| [[Angioimmunoblastic lymphoma]]
| size of cells ?
| site ?
| histomorphology ?
| IHC ?
| translocations ?
| clinical ?
| prevalence ?
| DDx ?
|-
| [[Enteropathy-type T cell lymphoma]]
| size of cells ?
| site ?
| histomorphology ?
| IHC ?
| translocations ?
| clinical ?
| prevalence ?
| DDx ?
|-
| Precursor lymphoblastic lymphoma / leukemia
| size of cells ?
| site ?
| histomorphology ?
| IHC ?
| translocations ?
| clinical ?
| prevalence ?
| DDx ?
|-
| [[Adult T-cell leukemia/lymphoma|Adult T-cell lymphoma / leukemia]]
| size of cells ?
| site ?
| cloverleaf nuclei (multilobular nuclei)
| IHC ?
| translocations ?
| poor prognosis ~ 1 year survival w/ Tx; d/t HTLV-1
| prevalence ?
| DDx ?
|-
| Peripheral T cell lymphoma (NOS)
| size of cells ?
| site ?
| histomorphology ?
| TIA-1+
| translocations ?
| clinical ?
| prevalence ?
| DDx ?
|-
| [[Anaplastic large cell lymphoma]]
| large
| deep & subcapsular sinuses of [[LN]]
| eosinophilic cytoplasm, nucleoli, often cohesive, wreath cell (C-shaped nucleus)
| CD30+/-, Alk+/-, CD4+, CD3-
| 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 ?
| uncommon
| carcinoma
|-
| [[Extranodal NK/T-cell lymphoma, nasal type|Extranodal NK/T cell lymphoma nasal type]]
| large ???
| nasal ???
| histomorphology ?
| EBER+, CD16+, CD56+, CD57-, TIA-1+, Granzyme B+
| translocations ?
| common in East Asia
| uncommon
| DDx ?
|-
| Blastic NK cell lymphoma
| size of cells ?
| site ?
| histomorphology ?
| IHC ?
| translocations ?
| clinical ?
| prevalence ?
| DDx ?
|}

==See also==
*[[Haematopathology]] - introduction.

==References==
{{reflist|2}}

[[Category:Haematopathology]]

Lymphoma

2025-07-13T21:02:54Z

Maxwell: /* Lymphoplasmacytic lymphoma */

[[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.

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

Other categorizations:
*T cell lymphomas (rare).
*B cell lymphomas (more common).

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

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

===Histologic classification===
#"Size".
#Nodularity.

===="Size"====
*The single most important factor for classifying lymphomas.
*Not really based on size.

{| class="wikitable"
|
| "Large"
| "Small"
| Utility
|-
| Nucleoli
| present
| absent
| most discriminative
|-
| Size
| >2x RBC dia.
| <2x RBC dia.
| moderate
|-
| Chromatin pattern
| "open" (pale)
| "closed"
| moderate/minimal
|-
| Cytoplasm
| mold-minimal <br>basophilic cytoplasm
| scant cytoplasm
| minimal
|}

====Histologic terms====
*Lymphomas = cells look discohesive, may be difficult to differentiate from poor differentiated carcinoma.
*[[Auer rods]] = [[acute myeloid leukemia]].
**Granular cytoplasmic rod (0.5-1 x4-6 micrometres).
**Not pathognomonic.
*Reed-Sternberg cells = [[Hodgkin's lymphoma]].
**Large cell - very large nucleus.
***Classically binucleated.
*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>
***''Mott cell'' is a cell that contains Russell bodies.<ref name=pmid3979421/>
*Effacement of nodal architecture.
*Loss of proliferation centers.

===IHC===
====General====
*CD45.
**AKA ''common lymphocyte antigen''.
**Useful to differentiate from carcinomas (e.g. small cell carcinoma).

Others:
*[[AE1/AE3]] -ve -- to r/o carcinoma.

====T cell markers====
*CD2 -- T cell marker (all T cells).
*CD3 -- T cell marker (all T cells).
**CD4 -- subset of T cells.
**CD8 -- subset of T cells.
*CD7 -- often lost first in T cell lymphomas.
*CD5 -- +ve in CLL & mantle cell lymphoma.
*CD43 -- +ve in mantle cell lymphoma

*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 - 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.
====B cell markers====
*CD20 -- B cell marker.
**CD19 -- B cell marker - used for [[flow cytometry]].
*PAX5 -- nuclear staining.<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/167414 http://www.ncbi.nlm.nih.gov/omim/167414]. Accessed on: 18 August 2010.</ref>
**Image: [http://www.pathologypics.com/PictView.aspx?ID=529 PAX5 in Hodgkin's lymphoma].
*CD79a.

*CD10 -- follicle center.
*BCL6 -- follicle center.
*BCL2 -- normally negative in germinal centers (GCs), marks T cell; +ve in small B cell lymphomas.
*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====
*CD23 -- 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====
=====Classic=====
*CD30 +ve -- Hodgkin's lymphoma (most sensitive).
*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==
{{main|Hodgkin's lymphoma}}
===General===
*Abbreviated ''HL''.

===Microscopic===
By definition, HL has Reed-Sternberg cells (RSCs).

====Classical HL====
Features (classic HL):
*Reed-Sternberg cell.
**Large binucleated cell.
***May be multinucleated.
***May have a horseshoe-like shape.
**Macronucleolus - approximately the size of a RBC (~8 micrometers).
**Well-defined cell border.

Notes:
*Large mononuclear cells are common (so called "mononuclear RSCs") but not diagnostic.

Images (classic HL):
*[http://commons.wikimedia.org/wiki/File:Hodgkin_lymphoma_cytology_large.jpg HL mixed cellularity - cytology (WC)].
*[http://commons.wikimedia.org/wiki/File:Hodgkin_lymphoma_cytology_small.jpg HL mixed cellularity - cytology (WC)].
*[http://en.wikipedia.org/wiki/File:Hodgkin_lymphoma_%281%29_mixed_cellulary_type.jpg HL mixed cellularity (WC)].

=====Subtypes=====
There are four CHL subtypes:<ref name=Ref_WMSP567>{{Ref WMSP|567}}</ref>
#Nodular sclerosis CHL - ~70% of CHL.
#*Mixed cellular background - T cell, plasma cells, eosinophils, neutrophils and histiocytes.
#*Nodular sclerosing fibrosis - thick strands fibrosis.
#Mixed cellularity CHL - ~20-25% of CHL.
#*Like nodular sclerosis - but no fibrosis.
#*May be associated with HIV infection.<ref name=pmid20138008>{{cite journal |author=Sissolak G, Sissolak D, Jacobs P |title=Human immunodeficiency and Hodgkin lymphoma |journal=Transfus. Apher. Sci. |volume=42 |issue=2 |pages=131–9 |year=2010 |month=April |pmid=20138008 |doi=10.1016/j.transci.2010.01.008 |url=}}</ref>
#Lymphocyte-rich CHL - rare.
#*T lymphocytes only (no mix of cells).
#Lymphocyte-depleted CHL - rare.
#*May be associated with [[HIV]] infection.<ref name=pmid20138008/>

Memory device:
*The subtypes prevalence is in reverse alphabetical order.

====Nodular lymphocyte-predominant HL====
Features (nodular lymphocyte-predominant Hodgkin's lymphoma):
*''Popcorn cell'' (previously known as ''Lymphocytic & histiocytic'' cell (L&H cell)<ref name=pmid9499174>{{cite journal |author=Küppers R, Rajewsky K, Braeuninger A, Hansmann ML |title=L&H cells in lymphocyte-predominant Hodgkin's disease |journal=N. Engl. J. Med. |volume=338 |issue=11 |pages=763–4; author reply 764–5 |year=1998 |month=March |pmid=9499174 |doi=10.1056/NEJM199803123381113 |url=}}</ref>) - variant of RSC:
**Cells (relatively) small (compared to classic RSCs).
**Lobulated nucleus - '''key feature'''.
**Small nucleoli.
*Subtle nodularity at low power (2.5x or 5x objective).

Images (NLPHL):
*[http://webpathology.com/image.asp?case=388&n=16 Popcorn cell (webpathology.com)].
*[http://commons.wikimedia.org/wiki/File:Popcorn_cell_in_nodular_lymphocyte_predominant_Hodgkin_lymphoma_-_very_high_mag_cropped.jpg Popcorn cell (WC)].

==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==
{{main|Diffuse large B cell lymphoma}}
===General===
*Abbreviated ''DLBCL''.

===Microscopic===
Features:<ref name=Ref_PBoD676>{{Ref PBoD|676 (???)}}</ref>
*Large cells -- 4-5 times the diameter of a small lymphocytes.
*Typically have marked cell-to-cell variation in size and shape.
*Cytoplasm usu. basophilic and moderate in abundance.
*+/-Prominent nucleoli, may be peripheral and/or multiple.

Notes:
*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==
{{main|Burkitt lymphoma}}
===General===
*Abbreviated ''BL''.
*Subtyped by etiology.

===Microscopic===
Features:
*"Starry-sky pattern":
**The ''stars'' in the pattern are: tingible-body macrophages.
***''Tingible-body macrophages'' = macrophages containing apoptotic tumour cells.
**The tumour cells are the sky.
*Tumour cells:<ref name=pmid12610094>{{cite journal |author=Bellan C, Lazzi S, De Falco G, Nyongo A, Giordano A, Leoncini L |title=Burkitt's lymphoma: new insights into molecular pathogenesis |journal=J. Clin. Pathol. |volume=56 |issue=3 |pages=188–92 |year=2003 |month=March |pmid=12610094 |pmc=1769902 |doi= |url=http://jcp.bmj.com/cgi/pmidlookup?view=long&pmid=12610094}}</ref>
**Medium-sized (~1.5-2x the size of a RBC) with uniform size ("monotonous") -- '''key feature'''.
**Round nucleus.
**Small nucleoli.
**Relatively abundant cytoplasm.
**Brisk mitotic rate.

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

==Plasma cell neoplasms==
{{Main|Plasma cell neoplasms}}
*These arise from ''plasma cells''.
*[[AKA]] ''plasma cell myeloma'', ''plasmacytoma''.
*''Multiple myeloma'' (a clinical diagnosis) fits into this category.

==Acute myeloid leukemia==
===General===
*May afflicits young adult.
*Males>females.

===Complications===
*[[Chloroma]] - soft tissue mass.
*Leukostasis.
**Occurs - lungs and brain.<ref>AML. Harrison's 16th Ed.</ref>
*Hyperviscosity syndrome.
*Spontaneous bleeding with low platelet counts.

===Classification===
There are two classifications:
#FAB (French-American-British) - based on histologic appearance/maturation.
#WHO classification.

===Histology===
*Auer rods - not required to diagnose.<ref>AG. 8 July, 2009.</ref>
**Cytoplasmic granular rods in blast cells.
***Dimensions: approx. 0.5-1 x 4-6 micrometres.
**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==
*Abbreviated ''EATL''.
*[[AKA]] ''enteropathy-type T-cell lymphoma'' (ETTL).
{{Main|Enteropathy-associated T-cell lymphoma}}

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

===Microscopic===
Features:
*Intermediate size cells with:
**+/-[[Vesicular nuclei]].
**Clear, moderate cytoplasm.
*"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===
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.
*TIA-1 -ve.

==Primary mediastinal B-cell lymphoma==
*Abbreviated ''PMBL''.
*[[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===
*Abbreviated as ''CTCL''.
**''Sézary syndrome'' is a subset of CTCL.

===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===
Features:
*Large cell lymphoma.

Images:
*[http://path.upmc.edu/cases/case695.html T-cell large granular lymphocytic leukemia (upmc.edu)].

===IHC===
*CD57 +ve -- '''important'''.
*CD3 +ve.
*CD5 +ve.
*CD45 +ve.

==Lymphoplasmacytic lymphoma==
:''Waldenström macroglobulinemia'' redirects here.
===General===
Features:<ref name=Ref_PCPBoD8_325>{{Ref PCPBoD8|325}}</ref>
*B cell neoplasm.
*Secretes monoclonal IgM.

Note:
*''Waldenström macroglobulinemia'' (WM) 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===
Features:<ref name=Ref_PCPBoD8_325>{{Ref PCPBoD8|325}}</ref>
*Plasmacytoid lymphocytes.
*Mixed inflammatory infiltrate with [[mast cell]]s, [[plasma cell]]s, lymphocytes.

DDx:
*[[Plasma cell neoplasm]].

===IHC===
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>
*PAX5 +ve.
*CD20 +ve.
*CD38 +ve.
*CD138 +ve.

Others:<ref name=pmid20654153/>
*CD5 -ve.
*CD10 -ve.
*CD23 -ve.
*CyclinD1 -ve.
*CD3 -ve.
*CD7 -ve.

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

===Microscopic===
Features:<ref name=Ref_PCPBoD8_328>{{Ref PCPBoD8|328}}</ref>
*Cloverleaf nuclei.
**Nuclei with multiple lobulations.

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}}

==Extranodal NK/T-cell lymphoma, nasal type==
*Abbreviated as ''ENKL'' or ''ENKTCL''.
*[[AKA]] ''extranodal natural kill lymphoma''
*[[AKA]] ''angiocentric lymphoma''.
{{Main|Extranodal NK/T-cell lymphoma, nasal type}}

==Table of lymphoma==
===B cell lymphomas===
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
! Name
! Size of cells
! Site
! Histomorphology
! IHC
! [[Translocations]]
! Clinical
! Prevalence
! DDx
|-
| [[Follicular lymphoma]]
| small (centrocytes)
| lymph node, germinal center
| abundant atypical follicles (lack GC polarity, lack polar mantle zone), effaced sinuses, scattered large cells (centroblasts)
| CD10+, bcl-6+<ref name=Ref_Lester95>{{Ref Lester|95}}</ref>
| t(14,18)
| usually indolent, may transform to DLBCL
| very common
| DLBCL, other small cell lymphomas
|-
| [[Mantle cell lymphoma]]
| small, monomorphic
| lymph node, mantle zone
| monomorphic lymphoid, abundant mitoses. +/-scattered epithelioid histiocytes, sclerosed blood vessels
| CD5+, CD23-, CD43+, cyclin D1+<ref name=Ref_Lester95>{{Ref Lester|95}}</ref>
| t(11;14)(q13;q32)<ref>URL: [http://atlasgeneticsoncology.org/Anomalies/t1114ID2021.html http://atlasgeneticsoncology.org/Anomalies/t1114ID2021.html]. Accessed on: 10 August 2010.</ref>
| indolent ???
| uncommon
| other [[small cell lymphomas]], [[PTGC]], [[Castleman disease]], [[Burkitt's lymphoma]]
|-
| Extranodal marginal zone lymphoma (MALT lymphoma)
| small
| mucosa-associated lymphoid tissue, GI tract + elsewhere
| +/- lymphepithelial lesion (cluster of 3+ cells in epithelium)
| CD21+, CD11c+, CD5-, CD23-<ref name=Ref_Lester95>{{Ref Lester|95}}</ref>
| ???
| indolent ???
| common
| other small cell lymphomas, neuroendocrine tumours
|-
| Precursor B-cell lymphoblastic lymphoma/ leukemia
| small
| 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>
| +/-t(12;21)<ref name=Ref_PCPBoD8_317>{{Ref PCPBoD8|317}}</ref>
| aggressive ???
| uncommon
| [[small cell lymphomas]]
|-
| [[Hairy cell leukemia]]
| small
| bone marrow, peripheral blood
| perinuclear clearing, clear cytoplasm, central nucleus
| CD25+, CD103+, CD5-<ref>URL: [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022&rendertype=table&id=A34029 http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022&rendertype=table&id=A34029]. Accessed on: 20 August 2010.</ref>
| translocations ?
| splenomegaly, no lymphadenopathy, pancytopenia, good prognosis with Tx
| uncommon
| small cell leukemias/lymphomas (e.g. [[SMZL]])
|-
| [[Burkitt's lymphoma]]
| medium, monomorphic
| lymph node, germinal center (???)
| tingible-body macrophages ("starry sky" appearance), round nucleus, small nucleoli, mitoses +++
| CD10+, BCL6+, BCL2-
| t(8;14) (q24;q32)
| rapid growth, may be associated with [[EBV]], [[HIV]]/AIDS
| uncommon
| DLBCL, mantle cell lymphoma
|-
| [[Diffuse large B cell lymphoma]]
| large cells (>2x RBC, often larger), variable size
| lymph node usually, germinal center
| sheets of large discohesive cells; if only nodular = follicular lymphoma
| MIB1 >40%
| none / like follicular lymphoma t(14,18) / c-MYC (like Burkitt lymphoma)
| poor prognosis
| very common
| Burkitt lymphoma, [[ALCL]], [[Hodgkin lymphoma]]
|-
| [[Primary mediastinal B-cell lymphoma]]
| large (>2x RBC, often larger), variable size
| mediastinum
| histomorphology ?
| IHC ?
| translocations ?
| predominantly young adults, better prognosis than [[DLBCL]]
| uncommon
| 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]]
|- 
|}

===T cell lymphomas===
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
! Name
! Size of cells
! Site
! Histomorphology
! IHC
! Translocations
! Clinical
! Prevalence
! DDx
|-
| [[Angioimmunoblastic lymphoma]]
| size of cells ?
| site ?
| histomorphology ?
| IHC ?
| translocations ?
| clinical ?
| prevalence ?
| DDx ?
|-
| [[Enteropathy-type T cell lymphoma]]
| size of cells ?
| site ?
| histomorphology ?
| IHC ?
| translocations ?
| clinical ?
| prevalence ?
| DDx ?
|-
| Precursor lymphoblastic lymphoma / leukemia
| size of cells ?
| site ?
| histomorphology ?
| IHC ?
| translocations ?
| clinical ?
| prevalence ?
| DDx ?
|-
| [[Adult T-cell leukemia/lymphoma|Adult T-cell lymphoma / leukemia]]
| size of cells ?
| site ?
| cloverleaf nuclei (multilobular nuclei)
| IHC ?
| translocations ?
| poor prognosis ~ 1 year survival w/ Tx; d/t HTLV-1
| prevalence ?
| DDx ?
|-
| Peripheral T cell lymphoma (NOS)
| size of cells ?
| site ?
| histomorphology ?
| TIA-1+
| translocations ?
| clinical ?
| prevalence ?
| DDx ?
|-
| [[Anaplastic large cell lymphoma]]
| large
| deep & subcapsular sinuses of [[LN]]
| eosinophilic cytoplasm, nucleoli, often cohesive, wreath cell (C-shaped nucleus)
| CD30+/-, Alk+/-, CD4+, CD3-
| 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 ?
| uncommon
| carcinoma
|-
| [[Extranodal NK/T-cell lymphoma, nasal type|Extranodal NK/T cell lymphoma nasal type]]
| large ???
| nasal ???
| histomorphology ?
| EBER+, CD16+, CD56+, CD57-, TIA-1+, Granzyme B+
| translocations ?
| common in East Asia
| uncommon
| DDx ?
|-
| Blastic NK cell lymphoma
| size of cells ?
| site ?
| histomorphology ?
| IHC ?
| translocations ?
| clinical ?
| prevalence ?
| DDx ?
|}

==See also==
*[[Haematopathology]] - introduction.

==References==
{{reflist|2}}

[[Category:Haematopathology]]

User:Maxwell

2025-07-13T20:34:01Z

Maxwell: Created page with "===Summary=== I am a 4th year medical student at Sidney Kimmel Medical College at Thomas Jefferson University, and I graduate in May 2026. I will be applying to combined Anato..."

===Summary===
I am a 4th year medical student at Sidney Kimmel Medical College at Thomas Jefferson University, and I graduate in May 2026.
I will be applying to combined Anatomic and Clinical Pathology (AP/CP) residency programs this application cycle (2025-2026).
My current pathology interests are cardiovascular pathology, hematopathology, neuropathology, general surgical pathology, microbiology, and forensic pathology. I am looking forward to exploring more in residency to figure out what fellowship path is best for me.

Prior to attending medical school, I worked as a software developer for Charles Schwab. My primary focus was automating software build and release processes.
I enjoy automating redundant processes, manipulating data, and running statistical tests.

Outside of school, I enjoy reading, trying new restaurants, and playing any sport. I currently play mostly roller hockey.