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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*[[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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==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=====
<gallery>
Image:Intravascular_lymphoma_-_high_mag.jpg | Intravascular lymphoma - high mag. (WC/Nephron)
Image:Intravascular_lymphoma_-_very_high_mag.jpg | Intravascular lymphoma - very high mag. (WC/Nephron)
</gallery>
www:
*[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)].
*[http://path.upmc.edu/cases/case473/images/fig01a.jpg ILBCL (upmc.edu)].<ref>URL: [http://path.upmc.edu/cases/case473.html http://path.upmc.edu/cases/case473.html]. Accessed on: 21 January 2012.</ref>
*[http://path.upmc.edu/cases/case697.html ILBCL - several images (upmc.edu)].
====IHC====
Features:<ref name=pmid11579120/>
*CD20 +ve - '''key feature'''.
*CD3 -ve.
*CD34 -ve.


==Burkitt's lymphoma==
==Burkitt's lymphoma==
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*Abbreviated ''EATL''.
*Abbreviated ''EATL''.
*[[AKA]] ''enteropathy-type T-cell lymphoma'' (ETTL).
*[[AKA]] ''enteropathy-type T-cell lymphoma'' (ETTL).
===General===
{{Main|Enteropathy-associated T-cell lymphoma}}
*T cell lymphoma due to [[celiac sprue]].
*Subdivided into type I and type II.<ref name=pmid21566094>{{cite journal |author=Delabie J, et al |title=Enteropathy-associated T-cell lymphoma: clinical and histological findings from the International Peripheral T-Cell Lymphoma Project|journal=Blood |volume=118|issue=148|pages= 148|year=2011|month=July}}</ref>
 
===Microscopic===
Features:<ref name=pmid21323966>{{Cite journal  | last1 = Takeshita | first1 = M. | last2 = Nakamura | first2 = S. | last3 = Kikuma | first3 = K. | last4 = Nakayama | first4 = Y. | last5 = Nimura | first5 = S. | last6 = Yao | first6 = T. | last7 = Urabe | first7 = S. | last8 = Ogawara | first8 = S. | last9 = Yonemasu | first9 = H. | title = Pathological and immunohistological findings and genetic aberrations of intestinal enteropathy-associated T cell lymphoma in Japan. | journal = Histopathology | volume = 58 | issue = 3 | pages = 395-407 | month = Feb | year = 2011 | doi = 10.1111/j.1365-2559.2011.03768.x | PMID = 21323966 }}</ref>
*Large ''or'' medium-sized lymphoid cells in the mucosa and submucosa.
**Large-sized cells ~ 2x RBC diameter = type 1.
**Medium sized cells ~ 1.5x RBC diameter = type 2.
*Intestinal epithelium usually preserved.
 
Image:
*[http://commons.wikimedia.org/wiki/File:Enteropathy-associated_T_cell_lymphoma_-_low_mag.jpg EATL (WC)].
 
===IHC===
Features - type 1:<ref name=pmid21323966/>
*CD56 +ve.
 
Features - type 2:<ref name=pmid21323966/><ref name=pmid21921780>{{Cite journal  | last1 = Chan | first1 = JK. | last2 = Chan | first2 = AC. | last3 = Cheuk | first3 = W. | last4 = Wan | first4 = SK. | last5 = Lee | first5 = WK. | last6 = Lui | first6 = YH. | last7 = Chan | first7 = WK. | title = Type II enteropathy-associated T-cell lymphoma: a distinct aggressive lymphoma with frequent γδ T-cell receptor expression. | journal = Am J Surg Pathol | volume = 35 | issue = 10 | pages = 1557-69 | month = Oct | year = 2011 | doi = 10.1097/PAS.0b013e318222dfcd | PMID = 21921780 }}</ref>
*CD3 +ve.
*CD5 -ve.
*CD8 -ve/+ve.
*CD56 -ve/+ve.
*CD30 +ve.


==Angioimmunoblastic T-cell lymphoma==
==Angioimmunoblastic T-cell lymphoma==
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Features:
Features:
*Intermediate size cells with:
*Intermediate size cells with:
**+/-Vesicular nuclei.
**+/-[[Vesicular nuclei]].
**Clear, moderate cytoplasm.
**Clear, moderate cytoplasm.
*"Empty" sinus; subcapsular sinuses "open".
*"Empty" sinus; subcapsular sinuses "open".
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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}}
===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>
*Rare.
*Young adults.
*[[Mediastinum]].
 
===Microscopic===
Features:
*Atypical large lymphoid cells - see ''[[DLBCL]]''.
*Classically associated with:
**Fibrosis
**Clear cells.
 
Note:
*Neither fibrosis or clear cells are required for the [[diagnosis]] nor are they pathognomonic.<ref name=pmid11251018 >{{Cite journal  | last1 = van Besien | first1 = K. | last2 = Kelta | first2 = M. | last3 = Bahaguna | first3 = P. | title = Primary mediastinal B-cell lymphoma: a review of pathology and management. | journal = J Clin Oncol | volume = 19 | issue = 6 | pages = 1855-64 | month = Mar | year = 2001 | doi =  | PMID = 11251018 }}</ref>
 
DDx:
*Other types of [[DLBCL]].
*Poorly differentiated carcinoma.
 
Images:
*[[WC]]:
**[http://commons.wikimedia.org/wiki/File:Primary_mediastinal_large_B-cell_lymphoma_-_very_high_mag.jpg PMBL - very high mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Primary_mediastinal_large_B-cell_lymphoma_-_intermed_mag.jpg PMBL - intermed. mag. (WC)].
*www:
**[http://path.upmc.edu/cases/case371.html PMBL - several images (upmc.edu)].
 
===Sign out===
<pre>
MASS, ANTERIOR MEDIASTINAL, CORE BIOPSY:
- LARGE B-CELL LYMPHOMA IN A FIBROTIC BACKGROUND WITH NECROSIS, SEE COMMENT
 
COMMENT:
Morphology:
Tumour cells: size ~2x a mature lymphocyte, a moderate quantity of grey/basophilic
  cytoplasm, no clear cells are identified.
Cells intermixed with tumour: mature lymphocytes, rare eosinophils.
Architecture: no gland formation, discohesive, no follicles apparent, extensive fibrosis.
 
Tumour cells:
POSITIVE: CD45, CD20, CD10, BCL-6, BCL-2.
NEGATIVE: pankeratin, PLAP, CD3, CD30.
Ki-67: 50% of large (lymphoid) cells.
</pre>


==Anaplastic large cell lymphoma==
==Anaplastic large cell lymphoma==
*Abbreviated ''ALCL''.
*Abbreviated ''ALCL''.
 
{{Main|Anaplastic large cell lymphoma}}
===General===
*May look a lot like a carcinoma.
**Often subcapsular in LNs.
*Usually T-cell derived.
*May be isolated to the [[skin]] - good prognosis.
 
Subtypes:
* Systemic ALCL.
* Cutaneous ALCL -- ALK -ve.
 
ALK IHC - systemic form:
* +ve = good prognosis.
* -ve = bad prognosis.
 
===Microscopic===
Features:
*Large cells with eosinophilic cytoplasm.
*Usually appear cohesive.
*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'''.
**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.
 
DDx:
*[[Hodgkin's lymphoma]].
*Carcinoma.
 
Images:
*[[WC]]:
**[http://commons.wikimedia.org/wiki/File:Anaplastic_large_cell_lymphoma_-_very_high_mag.jpg ALCL - very high mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Anaplastic_large_cell_lymphoma_-_cropped_-_very_high_mag.jpg ALCL - cropped - very high mag. (WC)].
*www:
**[http://path.upmc.edu/cases/case363.html ALCL - several images (upmc.edu)].
**[http://path.upmc.edu/cases/case557.html Small cell variant of ALCL - several images (upmc.edu)].
 
===IHC===
Features:
*CD30 +ve.
*ALK-1 -ve/+ve; strongly supports ALCL Dx if +ve.
*CD45 +ve.
*CD4 +ve.
*CD3 -ve/+ve.
*CD7 -ve/+ve.
*EMA +ve.
 
===Molecular===
*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.


==Cutaneous T cell lymphoma==
==Cutaneous T cell lymphoma==
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===IHC===
===IHC===
*CF57 +ve.
*CD57 +ve -- '''important'''.
*CD3 +ve.
*CD5 +ve.
*CD45 +ve.


==Lymphoplasmacytic lymphoma==
==Lymphoplasmacytic lymphoma==
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*[[AKA]] ''extranodal natural kill lymphoma''
*[[AKA]] ''extranodal natural kill lymphoma''
*[[AKA]] ''angiocentric lymphoma''.
*[[AKA]] ''angiocentric lymphoma''.
 
{{Main|Extranodal NK/T-cell lymphoma, nasal type}}
===General===
*Common in east Asia.<ref name=pmid18283711>{{Cite journal  | last1 = Suzuki | first1 = R. | last2 = Takeuchi | first2 = K. | last3 = Ohshima | first3 = K. | last4 = Nakamura | first4 = S. | title = Extranodal NK/T-cell lymphoma: diagnosis and treatment cues. | journal = Hematol Oncol | volume = 26 | issue = 2 | pages = 66-72 | month = Jun | year = 2008 | doi = 10.1002/hon.847 | PMID = 18283711 }}</ref>
*Strong association with [[EBV]].
 
===Microscopic===
Features:
*Atypical lymphoid cells:
**[[Nucleoli]].
**[[Nuclear pleomorphism]].
**Cells centered around vessels (angiocentric).
***May destroy small and medium sized arterioles.
 
Images:
*[http://path.upmc.edu/cases/case231/micro.html Extranodal NK/T-cell lymphoma, nasal type - case 1 - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case310.html Extranodal NK/T-cell lymphoma, nasal type - case 2 - several images (upmc.edu)].
 
===IHC===
Features:<ref name=pmid17064952>{{Cite journal  | last1 = Al-Hakeem | first1 = DA. | last2 = Fedele | first2 = S. | last3 = Carlos | first3 = R. | last4 = Porter | first4 = S. | title = Extranodal NK/T-cell lymphoma, nasal type. | journal = Oral Oncol | volume = 43 | issue = 1 | pages = 4-14 | month = Jan | year = 2007 | doi = 10.1016/j.oraloncology.2006.03.011 | PMID = 17064952 }}</ref>
*CD2 +ve.
*CD3 +ve (cytoplasmic).
*CD56 +ve.
 
Others:<ref>URL: [http://path.upmc.edu/cases/case310.html http://path.upmc.edu/cases/case310.html]. Accessed on: 14 January 2012.</ref>
*TIA-1 +ve.
*CD7 +ve.
*EBER +ve.


==Table of lymphoma==
==Table of lymphoma==
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