Burkitt's lymphoma pathophysiology: Difference between revisions
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{{CMG}};{{SM}}, {{AS}} | {{CMG}};{{SM}}, {{AS}} | ||
==Overview== | ==Overview== | ||
On microscopic histopathological analysis, "starry sky" appearance is characteristic finding of Burkitt's lymphoma. | |||
==Genetics== | ==Genetics== | ||
All types of Burkitt's lymphoma are characterized by dis regulation of the [[c-myc]] gene by one of three chromosomal [[translocations]].<ref name=Hoffman>{{cite book|last=Hoffman|first=Ronald|title=Hematology : basic principles and practice|year=2009|publisher=Churchill Livingstone/Elsevier|location=Philadelphia, PA|isbn=978-0-443-06715-0|pages=1304–1305|url=http://www.mdconsult.com/das/book/pdf/331595517-4/978-0-443-06715-0/4-u1.0-B978-0-443-06715-0..50083-2..DOCPDF.pdf?isbn=978-0-443-06715-0&eid=4-u1.0-B978-0-443-06715-0..50083-2..DOCPDF|edition=5th ed.}}</ref> This gene is found at [[chromosome 8|8q24]]. | All types of Burkitt's lymphoma are characterized by dis regulation of the ''[[c-myc]]''1 gene by one of three chromosomal [[translocations]].<ref name=Hoffman>{{cite book|last=Hoffman|first=Ronald|title=Hematology : basic principles and practice|year=2009|publisher=Churchill Livingstone/Elsevier|location=Philadelphia, PA|isbn=978-0-443-06715-0|pages=1304–1305|url=http://www.mdconsult.com/das/book/pdf/331595517-4/978-0-443-06715-0/4-u1.0-B978-0-443-06715-0..50083-2..DOCPDF.pdf?isbn=978-0-443-06715-0&eid=4-u1.0-B978-0-443-06715-0..50083-2..DOCPDF|edition=5th ed.}}</ref> This gene is found at [[chromosome 8|8q24]]. | ||
* The most common variant is t(8;14)(q24;q32), which accounts for approximately 85%<ref name=Hoffman /> of cases. This involves [[c-myc]] and [[IGH@]]. A variant of this, a three-way translocation, t(8;14;18), has also been identified.<ref name="pmid17547754">{{cite journal |author=Liu D, Shimonov J, Primanneni S, Lai Y, Ahmed T, Seiter K |title=t(8;14;18): a 3-way chromosome translocation in two patients with Burkitt's lymphoma/leukemia |journal=Mol. Cancer |volume=6 |issue= |pages=35 |year=2007 |pmid=17547754 |doi=10.1186/1476-4598-6-35 |pmc=1904237}}</ref> | *1. The most common variant is t(8;14)(q24;q32), which accounts for approximately 85%<ref name=Hoffman /> of cases. This involves [[c-myc]] and [[IGH@]]. A variant of this, a three-way translocation, t(8;14;18), has also been identified.<ref name="pmid17547754">{{cite journal |author=Liu D, Shimonov J, Primanneni S, Lai Y, Ahmed T, Seiter K |title=t(8;14;18): a 3-way chromosome translocation in two patients with Burkitt's lymphoma/leukemia |journal=Mol. Cancer |volume=6 |issue= |pages=35 |year=2007 |pmid=17547754 |doi=10.1186/1476-4598-6-35 |pmc=1904237}}</ref> | ||
* A rare variant is at t(2;8)(p12;q24).<ref name="pmid18813817">{{cite journal |author=Smardova J, Grochova D, Fabian P, ''et al.'' |title=An unusual p53 mutation detected in Burkitt's lymphoma: 30 bp duplication |journal=Oncol. Rep. |volume=20 |issue=4 |pages=773–8 |year=2008 |month=October |pmid=18813817 |doi= |url=http://www.spandidos-publications.com/or/article.jsp?article_id=or_20_4_773}}</ref> This involves [[IGK@]] and c-myc. | *2. A rare variant is at t(2;8)(p12;q24).<ref name="pmid18813817">{{cite journal |author=Smardova J, Grochova D, Fabian P, ''et al.'' |title=An unusual p53 mutation detected in Burkitt's lymphoma: 30 bp duplication |journal=Oncol. Rep. |volume=20 |issue=4 |pages=773–8 |year=2008 |month=October |pmid=18813817 |doi= |url=http://www.spandidos-publications.com/or/article.jsp?article_id=or_20_4_773}}</ref> This involves [[IGK@]] and c-myc. | ||
* Another rare variant is t(8;22)(q24;q11).<ref name="pmid18813817"/> This involves [[IGL@]] and c-myc. | *3. Another rare variant is t(8;22)(q24;q11).<ref name="pmid18813817"/> This involves [[IGL@]] and c-myc. | ||
Combined, the two less-common translocations, t(2;8)(p12;q24) and t(8;22)(q24;q11), account for the remaining 15% of cases not due to the t(8;14)(q24;q32) translocation.<ref name=Hoffman /> | Combined, the two less-common translocations, t(2;8)(p12;q24) and t(8;22)(q24;q11), account for the remaining 15% of cases not due to the t(8;14)(q24;q32) translocation.<ref name=Hoffman /> | ||
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==Microscopic Pathology== | ==Microscopic Pathology== | ||
The tumor consists of sheets of a monotonous (i.e. similar in size and morphology) population of medium size lymphoid cells with high proliferative activity and [[apoptotic]] activity. The "starry sky" appearance seen<ref name="pmid15086279">{{cite journal |author=Fujita S, Buziba N, Kumatori A, Senba M, Yamaguchi A, Toriyama K |title=Early stage of Epstein-Barr virus lytic infection leading to the "starry sky" pattern formation in endemic Burkitt lymphoma |journal=Arch. Pathol. Lab. Med. |volume=128 |issue=5 |pages=549–52 |year=2004 |month=May |pmid=15086279 |doi= 10.1043/1543-2165(2004)128<549:ESOEVL>2.0.CO;2|url=http://journals.allenpress.com/jrnlserv/?request=get-abstract&issn=0003-9985&volume=128&page=549}}</ref> under low power is due to scattered tingible body-laden [[macrophages]] (macrophages containing dead body of apoptotic tumor cells). The old descriptive term of "small non-cleaved cell" is misleading. The tumor cells are mostly medium in size (i.e. tumor nuclei size similar to that of [[histiocytes]] or [[endothelial cells]]). "Small non-cleaved cells" are compared to "large non-cleaved cells" of normal germinal center lymphocytes. Tumor cells possess small amount of [[basophilic]] cytoplasm. The cellular outline usually appears squared off. Shown below is a micrograph of touch prep stained with [[Wright's stain]]. | The tumor consists of sheets of a monotonous (i.e. similar in size and morphology) population of medium size lymphoid cells with high proliferative activity and [[apoptotic]] activity. The "starry sky" appearance seen<ref name="pmid15086279">{{cite journal |author=Fujita S, Buziba N, Kumatori A, Senba M, Yamaguchi A, Toriyama K |title=Early stage of Epstein-Barr virus lytic infection leading to the "starry sky" pattern formation in endemic Burkitt lymphoma |journal=Arch. Pathol. Lab. Med. |volume=128 |issue=5 |pages=549–52 |year=2004 |month=May |pmid=15086279 |doi= 10.1043/1543-2165(2004)128<549:ESOEVL>2.0.CO;2|url=http://journals.allenpress.com/jrnlserv/?request=get-abstract&issn=0003-9985&volume=128&page=549}}</ref> under low power is due to scattered tingible body-laden [[macrophages]] (macrophages containing dead body of apoptotic tumor cells). The old descriptive term of "small non-cleaved cell" is misleading. The tumor cells are mostly medium in size (i.e. tumor nuclei size similar to that of [[histiocytes]] or [[endothelial cells]]). "Small non-cleaved cells" are compared to "large non-cleaved cells" of normal germinal center lymphocytes. Tumor cells possess small amount of [[basophilic]] cytoplasm. The cellular outline usually appears squared off. Shown below is a micrograph of touch prep stained with [[Wright's stain]]. | ||
[[Image:800px-Burkitt_lymphoma,_touch_prep,_Wright_stain.jpg|thumb|none|200px|Burkitt lymphoma, touch prep, | [[Image:800px-Burkitt_lymphoma,_touch_prep,_Wright_stain.jpg|thumb|none|200px|Burkitt lymphoma, touch prep, wright stain]] | ||
===Video=== | ===Video=== | ||
Revision as of 14:52, 1 October 2015
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Shivali Marketkar, M.B.B.S. [2], Sowminya Arikapudi, M.B,B.S. [3]
Overview
On microscopic histopathological analysis, "starry sky" appearance is characteristic finding of Burkitt's lymphoma.
Genetics
All types of Burkitt's lymphoma are characterized by dis regulation of the c-myc1 gene by one of three chromosomal translocations.[1] This gene is found at 8q24.
- 1. The most common variant is t(8;14)(q24;q32), which accounts for approximately 85%[1] of cases. This involves c-myc and IGH@. A variant of this, a three-way translocation, t(8;14;18), has also been identified.[2]
- 2. A rare variant is at t(2;8)(p12;q24).[3] This involves IGK@ and c-myc.
- 3. Another rare variant is t(8;22)(q24;q11).[3] This involves IGL@ and c-myc.
Combined, the two less-common translocations, t(2;8)(p12;q24) and t(8;22)(q24;q11), account for the remaining 15% of cases not due to the t(8;14)(q24;q32) translocation.[1]
Immunohistochemistry
The tumor cells in Burkitt lymphoma generally strongly express markers of B cell differentiation (CD20, CD22, CD19) as well as CD10, and BCL6. The tumor cells are generally negative for BCL2 and TdT. The high mitotic activity of Burkitt lymphoma is confirmed by nearly 100% of the cells staining positive for Ki67.[4]
Malignant B cell characteristics
Malignant B cells have identical DNA recombinations of the V(D)J region of the immunoglobin genes. This means that no increase in specificity of antibody molecules is occurring in the malignant cells. These malignant cells are thus clonal populations and can be assayed for by using DNA probes specific for the regions where recombination is expected. Normal DNA will be characterized by two high concentration of identical germ line DNA V (D) J regions and endless, likely undetectable, non-germline Ig V(D)J DNA. Lymphoma cells have an additional high concentration of V(D)J DNA that is unlike the germ line, indicating clonal populations of B Cells that are not undifferentiated B cells (germ line DNA cells). Assays typically use the process of electrophoresis and southern blot analysis to determine the existence of these characteristics.
Gross Pathology
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Burkitt lymphoma: bilateral eye involvement -
Seven-year-old Nigerian boy with a several-month history of jaw swelling which had been treated with antibiotics: The tumor was ulcerated and draining -
Picture of a mouth of a patient with Burkitt lymphoma showing disruption of teeth and partial obstruction of airway
Microscopic Pathology
The tumor consists of sheets of a monotonous (i.e. similar in size and morphology) population of medium size lymphoid cells with high proliferative activity and apoptotic activity. The "starry sky" appearance seen[5] under low power is due to scattered tingible body-laden macrophages (macrophages containing dead body of apoptotic tumor cells). The old descriptive term of "small non-cleaved cell" is misleading. The tumor cells are mostly medium in size (i.e. tumor nuclei size similar to that of histiocytes or endothelial cells). "Small non-cleaved cells" are compared to "large non-cleaved cells" of normal germinal center lymphocytes. Tumor cells possess small amount of basophilic cytoplasm. The cellular outline usually appears squared off. Shown below is a micrograph of touch prep stained with Wright's stain.
Video
{{#ev:youtube|CwsQ-wIbbK8}}
References
- ↑ 1.0 1.1 1.2 Hoffman, Ronald (2009). Hematology : basic principles and practice (PDF) (5th ed. ed.). Philadelphia, PA: Churchill Livingstone/Elsevier. pp. 1304–1305. ISBN 978-0-443-06715-0.
- ↑ Liu D, Shimonov J, Primanneni S, Lai Y, Ahmed T, Seiter K (2007). "t(8;14;18): a 3-way chromosome translocation in two patients with Burkitt's lymphoma/leukemia". Mol. Cancer. 6: 35. doi:10.1186/1476-4598-6-35. PMC 1904237. PMID 17547754.
- ↑ 3.0 3.1 Smardova J, Grochova D, Fabian P; et al. (2008). "An unusual p53 mutation detected in Burkitt's lymphoma: 30 bp duplication". Oncol. Rep. 20 (4): 773–8. PMID 18813817. Unknown parameter
|month=ignored (help) - ↑ . ISBN 978-92-832-2431-0. Missing or empty
|title=(help) - ↑ Fujita S, Buziba N, Kumatori A, Senba M, Yamaguchi A, Toriyama K (2004). "Early stage of Epstein-Barr virus lytic infection leading to the "starry sky" pattern formation in endemic Burkitt lymphoma". Arch. Pathol. Lab. Med. 128 (5): 549–52. doi:10.1043/1543-2165(2004)128<549:ESOEVL>2.0.CO;2. PMID 15086279. Unknown parameter
|month=ignored (help)