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Difference between revisions of "Burkitt lymphoma"
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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Burkitt_lymphoma,_H%26E.jpg | |||
| Width = | |||
| Caption = Starry-sky pattern. [[H&E stain]]. | |||
| Micro = medium-sized lymphoid cells of uniform size with abundant cytoplasm, interspersed macrophages | |||
| Subtypes = endemic, non-endemic, immunodeficiency associated | |||
| LMDDx = [[DLBCL]], intermediate DLBCL/BL | |||
| Stains = | |||
| IHC = [[CD20]]+, CD10+, BCL6+, [[EBER]]+, Ki-67 (100%) | |||
| EM = | |||
| Molecular = t(8;14) (q24;q32) + others | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Site = [[lymph node]] | |||
| Assdx = +/-[[EBV]] infection, +/-[[HIV]] infection | |||
| Syndromes = | |||
| Clinicalhx = very rapid growth | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = uncommon | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = | |||
| Other = | |||
| ClinDDx = | |||
}} | |||
'''Burkitt lymphoma''', abbreviated '''BL''', is an uncommon [[lymphoma]] with medium-sized cells. | '''Burkitt lymphoma''', abbreviated '''BL''', is an uncommon [[lymphoma]] with medium-sized cells. | ||
==General== | ==General== | ||
*Extremely high proliferative rate & rate of apoptosis. | *Extremely high proliferative rate & rate of [[apoptosis]] | ||
*Rare in adults -- 1-2% of adult lymphomas.<ref name=omim113970>{{OMIM|113970}}</ref> | |||
*Relatively common in children -- 30-50% of childhood lymphomas.<ref name=omim113970>{{OMIM|113970}}</ref> | |||
*Although aggressive untreated, the high mitotic activity makes it relatively chemosensitive and complete remissions of 75-90% are described | |||
===Pathophysiology=== | |||
*Origin cell: germinal centre B cells (favoured) vs. memory B cells.<ref name=pmid12610094/> | |||
==Subtypes== | ==Subtypes== | ||
| Line 8: | Line 43: | ||
#Endemic: | #Endemic: | ||
#*Found in Africa. | #*Found in Africa. | ||
#*EBV (Epstein-Barr virus) associated.<ref name=pmid12610094/> | #*EBV ([[Epstein-Barr virus]]) associated.<ref name=pmid12610094/> | ||
#Non-endemic: | #Non-endemic/sporadic: | ||
#*Typical of the BL seen in the western world; EBV negative. | #*Typical of the BL seen in the western world; EBV negative. | ||
#Immunodeficiency associated: | #Immunodeficiency associated: | ||
#*Associated with HIV infection. | #*Associated with [[HIV]] infection. | ||
#*Post-transplantation immunosuppression | |||
* | |||
==Microscopic== | ==Microscopic== | ||
Features: | Features: | ||
*"Starry-sky pattern" | *"Starry-sky pattern": | ||
**The ''stars'' in the pattern are: tingible- | **The ''stars'' in the pattern are: tingible-body macrophages. | ||
***'' | ***''Tingible-body macrophages'' = macrophages containing apoptotic tumour cells. | ||
**The tumour cells are the sky. | **The tumour cells are the sky. | ||
*Tumour cells:<ref name=pmid12610094/> | *Tumour cells:<ref name=pmid12610094/> | ||
**Medium | **Medium-sized (~1.5-2x the size of a RBC) with uniform size ("monotonous") -- '''key feature'''. | ||
**Round | **Round nucleus. | ||
**Small | **Small nucleoli. | ||
**Relatively abundant cytoplasm. | **Relatively abundant cytoplasm. | ||
**Brisk mitotic rate. | **Brisk mitotic rate (near 100%). | ||
DDx: | |||
*[[DLBCL]]. | |||
*B cell lymphoma unclassifiable with features intermediate between DLBCL and Burkitt lymphoma.<ref name=pmid20398809>{{Cite journal | last1 = Carbone | first1 = A. | last2 = Gloghini | first2 = A. | last3 = Aiello | first3 = A. | last4 = Testi | first4 = A. | last5 = Cabras | first5 = A. | title = B-cell lymphomas with features intermediate between distinct pathologic entities. From pathogenesis to pathology. | journal = Hum Pathol | volume = 41 | issue = 5 | pages = 621-31 | month = May | year = 2010 | doi = 10.1016/j.humpath.2009.10.027 | PMID = 20398809 }}</ref> | |||
**May be referred to as ''intermediate DLBCL/BL''.<ref name=pmid22389885>{{Cite journal | last1 = Ahn | first1 = JY. | last2 = Seo | first2 = YH. | last3 = Park | first3 = PW. | last4 = Kim | first4 = KH. | last5 = Park | first5 = MJ. | last6 = Jeong | first6 = JH. | last7 = Park | first7 = SH. | last8 = Song | first8 = YH. | title = A case of B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma in a Korean child. | journal = Ann Lab Med | volume = 32 | issue = 2 | pages = 162-6 | month = Mar | year = 2012 | doi = 10.3343/alm.2012.32.2.162 | PMID = 22389885 }}</ref> | |||
===Images=== | |||
<gallery> | |||
Image:Burkitt_lymphoma,_H%26E.jpg | Starry-sky pattern. (WC/Uthman) | |||
</gallery> | |||
www: | |||
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1769902/figure/f1/ Burkitt lymphoma (nih.gov)]. | |||
==Cytology== | |||
Features: | |||
*Medium-sized lymphoid cells with small lipid vacuoles (~1-2 micrometers).<ref name=pmid15986398>{{Cite journal | last1 = Troxell | first1 = ML. | last2 = Bangs | first2 = CD. | last3 = Cherry | first3 = AM. | last4 = Natkunam | first4 = Y. | last5 = Kong | first5 = CS. | title = Cytologic diagnosis of Burkitt lymphoma. | journal = Cancer | volume = 105 | issue = 5 | pages = 310-8 | month = Oct | year = 2005 | doi = 10.1002/cncr.21307 | PMID = 15986398 }}</ref> | |||
Image: [http:// | Image: | ||
*[http://commons.wikimedia.org/wiki/File:Burkitt_lymphoma,_touch_prep,_Wright_stain.jpg Burkitt lymphoma (WC/Uthman)]. | |||
==IHC== | ==IHC== | ||
Features: | Features: | ||
*CD20 +ve. | *[[CD20]] +ve. | ||
*CD10 +ve. | *CD10 +ve. | ||
*BCL6 +ve. | *BCL6 +ve. | ||
*EBER +ve. | *[[EBER]] +ve. | ||
*Ki-67 ~100%. | |||
Others: | Others: | ||
*BCL2 -ve. | *BCL2 -ve (may show weak BCL2 | ||
==Molecular pathology== | |||
*Isolated CMYC-IGH [[translocation]] - t(8;14) (q24;q32).<ref name=pmid12610094/> | |||
*Several other variants -- involve MYC rearrangement with other partners.<ref name=pmid12610094/><ref name=omim113970>{{OMIM|113970}}</ref> | |||
*Should not have CCND1, BCL6 or BCL2 translocations | |||
Note: | |||
* | *MYC rearrangement is typically assessed with an [[ISH]] break apart probe. | ||
==See also== | ==See also== | ||
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[[Category:Haematopathology]] | [[Category:Haematopathology]] | ||
[[Category:Diagnosis]] | |||