Difference between revisions of "Diffuse large B-cell lymphoma"

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Diffuse large B-cell lymphoma (view source)

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'''Diffuse large B cell lymphoma''', abbreviated '''DLBCL''', is a very common [[lymphoma]] with a poor prognosis.
{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image = Diffuse_large_B_cell_lymphoma_-_cytology_low_mag.jpg
| Width =
| Caption = DLBCL - cytology.
| Micro = large lymphoid cells (>=2x resting lymphocyte), nucleolus, sheeting architecture
| Subtypes =
| LMDDx = [[follicular lymphoma]], [[Hodgkin lymphoma]], [[PTLD]], primary effusion lymphoma, carcinoma
| Stains =
| IHC = CD20+, CD10+/-, BCL6+/-, CD45+
| EM =
| Molecular = t(14;18)(q32;q21)/IGH-BCL2 - esp. when arising from follicular lymphoma
| IF =
| Gross =
| Grossing =
| Site = [[Lymph node]]
| Assdx =
| Syndromes =
| Clinicalhx =
| Signs =
| Symptoms =
| Prevalence =
| Bloodwork = +/- cytopenia
| Rads = lymphadenopathy
| Endoscopy =
| Prognosis = poor
| Other =
| ClinDDx = metastatic carcinoma, reactive lymphadenopathy, infective lymphadenopathy}}
'''Diffuse large B-cell lymphoma''', abbreviated '''DLBCL''', is a very common [[lymphoma]] with a poor prognosis.


==General==
==General==
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*May arise from [[follicular lymphoma]].
*May arise from [[follicular lymphoma]].
*Most common form of non-Hodgkin lymphoma (in the USA).<ref name=Ref_PCPBoD8_321>{{Ref PCPBoD8|321}}</ref>
*Most common form of non-Hodgkin lymphoma (in the USA).<ref name=Ref_PCPBoD8_321>{{Ref PCPBoD8|321}}</ref>
*Most common lymphoma of the [[spleen]].<ref name=pmid22924843>{{Cite journal  | last1 = Shimizu-Kohno | first1 = K. | last2 = Kimura | first2 = Y. | last3 = Kiyasu | first3 = J. | last4 = Miyoshi | first4 = H. | last5 = Yoshida | first5 = M. | last6 = Ichikawa | first6 = R. | last7 = Niino | first7 = D. | last8 = Ohshima | first8 = K. | title = Malignant lymphoma of the spleen in Japan: a clinicopathological analysis of 115 cases. | journal = Pathol Int | volume = 62 | issue = 9 | pages = 577-82 | month = Sep | year = 2012 | doi = 10.1111/j.1440-1827.2012.02844.x | PMID = 22924843 }}</ref>
*Obesity is a risk factor.<ref>{{Cite journal  | last1 = Castillo | first1 = JJ. | last2 = Ingham | first2 = RR. | last3 = Reagan | first3 = JL. | last4 = Furman | first4 = M. | last5 = Dalia | first5 = S. | last6 = Mitri | first6 = J. | title = Obesity is associated with increased relative risk of diffuse large B-cell lymphoma: a meta-analysis of observational studies. | journal = Clin Lymphoma Myeloma Leuk | volume = 14 | issue = 2 | pages = 122-30 | month = Apr | year = 2014 | doi = 10.1016/j.clml.2013.10.005 | PMID = 24360912 }}</ref>
*Suscepibility loci located on 6p, 2p and 8q.<ref>{{Cite journal  | last1 = Cerhan | first1 = JR. | last2 = Berndt | first2 = SI. | last3 = Vijai | first3 = J. | last4 = Ghesquières | first4 = H. | last5 = McKay | first5 = J. | last6 = Wang | first6 = SS. | last7 = Wang | first7 = Z. | last8 = Yeager | first8 = M. | last9 = Conde | first9 = L. | title = Genome-wide association study identifies multiple susceptibility loci for diffuse large B cell lymphoma. | journal = Nat Genet | volume = 46 | issue = 11 | pages = 1233-8 | month = Nov | year = 2014 | doi = 10.1038/ng.3105 | PMID = 25261932 }}</ref>


==Microscopic==
==Microscopic==
Features:<ref name=Ref_PCPBoD8_321>{{Ref PCPBoD8|321}}</ref><ref name=Ref_PBoD676>{{Ref PBoD|676 (???)}}</ref>
Features:<ref name=Ref_PCPBoD8_321>{{Ref PCPBoD8|321}}</ref><ref name=Ref_PBoD676>{{Ref PBoD|676 (???)}}</ref>
*Large lymphoid cells:
*Large lymphoid cells:
**4-5x the diameter of a small lymphocytes.
**>= 2x the diameter of a small lymphocytes.
**Marked cell-to-cell variation in size and shape.  
**Marked cell-to-cell variation in size and shape.  
**Cytoplasm usually basophilic and moderate in abundance.  
**Cytoplasm usually basophilic and moderate in abundance.  
**+/-Prominent nucleoli, may be peripheral and/or multiple.  
**+/-Prominent nucleoli, may be peripheral and/or multiple.  
*'''Not''' follicular - nodular arrangement.
*'''Not''' follicular/nodular arrangement.
**Follicular arrangement = [[follicular lymphoma]].
**Follicular arrangement = [[follicular lymphoma]].


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*Large bizarre cells can occasionally mimic Reed-Sternberg cells, seen in [[Hodgkin lymphoma]].
*Large bizarre cells can occasionally mimic Reed-Sternberg cells, seen in [[Hodgkin lymphoma]].


Images:
===Differential diagnosis===
*[http://commons.wikimedia.org/wiki/File:Diffuse_large_B_cell_lymphoma_-_cytology_low_mag.jpg DLBCL - cytology (WC)].
*[[Carcinoma]] - esp. [[small cell carcinoma]].
*[[Anaplastic large cell lymphoma]] (ALCL).
*[[Follicular lymphoma]].
**If a nodular architecture is present it is follicular lymphoma.
*[[Post-transplant lymphoproliferative disorder]] (PTLD) - in organ transplant recipients.
*Primary effusion lymphoma - seen in [[HIV]] infections.
*Mixed cellularity [[Hodgkin lymphoma]] - esp. for T-cell/histiocyte-rich large B cell lymphoma.
*B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma.<ref name=pmid22222636>{{Cite journal  | last1 = Gualco | first1 = G. | last2 = Natkunam | first2 = Y. | last3 = Bacchi | first3 = CE. | title = The spectrum of B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma: a description of 10 cases. | journal = Mod Pathol | volume =  | issue =  | pages =  | month = Jan | year = 2012 | doi = 10.1038/modpathol.2011.200 | PMID = 22222636 | URL = http://www.nature.com/modpathol/journal/vaop/ncurrent/full/modpathol2011200a.html }}</ref>
*Other [[small round cell tumours]].


===Images===
<gallery>
Image: Diffuse large B cell lymphoma (1) tonsil.jpg | DLBCL. (WC/KGH)
Image: Diffuse large B cell lymphoma (2) tonsil.jpg | DLBCL. (WC/KGH)
Image: Diffuse large B cell lymphoma (3) tonsil.jpg | DLBCL. (WC/KGH)
</gallery>
<gallery>
Image: DLBCL of testis - 2 -- intermed mag.jpg | DLBCL (testis) - intermed. mag. (WC)
Image: DLBCL of testis - 2 -- high mag.jpg | DLBCL (testis) - high mag. (WC)
Image: DLBCL of testis - 2 -- very high mag.jpg | DLBCL (testis) - very high mag. (WC)
</gallery>
<gallery>
Image:Diffuse_large_B_cell_lymphoma_-_cytology_low_mag.jpg | DLBCL - cytology (WC)
</gallery>


==Special DLBCL subtypes==
===Special subtypes of diffuse large B-cell lymphoma===
*[[Primary mediastinal B-cell lymphoma]] (PMBL) - esp. in young adults.
*[[Primary mediastinal B-cell lymphoma]] (PMBL) - esp. in young adults.
*T-cell/histiocyte-rich large B cell lymphoma.
*T-cell/histiocyte-rich large B cell lymphoma.
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*Plasmablastic lymphoma.
*Plasmablastic lymphoma.
*LBCL arising in [[HHV-8]] associated multicentric [[Castleman disease]].
*LBCL arising in [[HHV-8]] associated multicentric [[Castleman disease]].
*Primary effusion lymphoma.
*Primary effusion lymphoma - associated with [[human immunodeficiency virus]].
*Lymphomatoid granulomatosis.
*Lymphomatoid granulomatosis.
==Differential diagnosis==
*Carcinoma.
*[[Anaplastic large cell lymphoma]] (ALCL).
*[[Follicular lymphoma]].
**If a nodular architecture is present it is follicular lymphoma.
*[[Post-transplant lymphoproliferative disorder]] (PTLD) - in organ transplant recipients.
*Primary effusion lymphoma - seen in [[HIV]] infections.
*Mixed cellularity [[Hodgkin lymphoma]] - esp. for T-cell/histiocyte-rich large B cell lymphoma.
*B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma.<ref name=pmid22222636>{{Cite journal  | last1 = Gualco | first1 = G. | last2 = Natkunam | first2 = Y. | last3 = Bacchi | first3 = CE. | title = The spectrum of B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma: a description of 10 cases. | journal = Mod Pathol | volume =  | issue =  | pages =  | month = Jan | year = 2012 | doi = 10.1038/modpathol.2011.200 | PMID = 22222636 | URL = http://www.nature.com/modpathol/journal/vaop/ncurrent/full/modpathol2011200a.html }}</ref>


==IHC==
==IHC==
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{{familytree/end}}
{{familytree/end}}


==="UHN panel"===
===A panel===
*H&E 2 micrometers.
*H&E 2 micrometers.
*CD20 -- B cells.
*CD20 -- B cells.
*CD10 -- follicular lymphoma.
*CD10 -- follicular lymphoma (membrane).
*BCL6 -- follicular lymphoma.
*BCL6 -- follicular lymphoma (nucleus).
*BCL2 -- follicular lymphoma.
*BCL2 -- follicular lymphoma (membrane/cytoplasm).
*MUM1 -- B cells.
*MUM1 -- B cells.
*CD21 -- highlight FDC networks if present.
*CD21 -- highlight FDC networks if present.
*CD23 -- highlight FDC networks if present.
*[[CD23]] -- highlight FDC networks if present.
*MIB1 -- proliferative rate.
*MIB1 -- proliferative rate.
*EBER -- [[EBV]].
*EBER -- [[EBV]].
*CD3 -- T cells.
*CD3 -- T cells (membrane/cytoplasm).
*CD5 -- T cells.
*CD5 -- T cells (membrane).
*Unstained x4.
*Unstained x4.


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COMMENT:
COMMENT:
The tumour consists of cells approximately 2x the size of mature lymphocytes.  Mature lymphocytes are intermixed with the tumour. There is no gland formation. Follicle formation is not apparent.
The tumour consists of cells approximately 2x the size of mature  
lymphocytes.  Mature lymphocytes are intermixed with the tumour.  
There is no gland formation. Follicle formation is not apparent.
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<pre>
SPLEEN, SPLENECTOMY:
- DIFFUSE LARGE B-CELL LYMPHOMA.
 
COMMENT:
Architecture: no gland formation, no follicle formation apparent, dyscohesive.
Tumour cell size: 1.5-2x resting lymphocyte.
Nucleoli: present.
Cytoplasm: scant-to-moderate basophilic/grey.
Proliferation: mitoses easily identified.
 
Positive stains (tumour cells):
CD45, CD20, BCL-2, BCL-6.
 
Negative stains (tumour cells):
pankeratin, S-100, MUM-1, CD3, CD5, CD10, CD21, CD30.
 
Ki-67: 20% of cells.
</pre>
</pre>


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