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{{Angioimmunoblastic T-cell lymphoma}}
{{Angioimmunoblastic T-cell lymphoma}}
{{CMG}}; {{AE}} {{RT}} {{AS}}
{{CMG}}; {{AE}} {{RT}} {{AS}}
==Pathophysiology==
==Overview==
===Genetics===
[[Clone|Clonal]] [[T-cell receptor]] and [[immunoglobulin]] [[gene]] rearrangements are involved in the [[pathogenesis]] of angioimmunoblastic T-cell lymphoma. On [[gross pathology]], aborization and proliferation of [[high endothelial venules]] are characteristic findings of angioimmunoblastic T-cell lymphoma. On [[microscopic]] [[Histopathology|histopathological]] [[analysis]], [[CD4]]+ [[T-cells]], [[CD8]]+ [[T-cells]], and polyclonal [[plasma cells]] are characteristic findings of angioimmunoblastic T-cell lymphoma.
Clonal [[T-cell receptor]] gene rearrangements are detected in 75% of cases<ref name="fell1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=2849301&query_hl=34&itool=pubmed_ExternalLink]
==Genetics==
Feller AC, Griesser H, Schilling CV, Wacker HH, Dallenbach F, Bartels H, Kuse R, Mak TW, Lennert K. "Clonal gene rearrangement patterns correlate with immunophenotype and clinical parameters in patients with angioimmunoblastic lymphadenopathy." '''Am J Pathol'''. 1988 Dec;133(3):549-56. PMID: 2849301</ref>, and [[immunoglobin]] gene rearrangements are seen in 10% of cases, and these cases are believed to be due to expanded EBV-driven [[B-cell]] populations.<ref name="lip1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=3805286&query_hl=36&itool=pubmed_ExternalLink]
* [[Clone|Clonal]] [[T-cell receptor]] [[gene]] rearrangements are detected in 75% of the cases.<ref name="fell1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=2849301&query_hl=34&itool=pubmed_ExternalLink]
Lipford EH, Smith HR, Pittaluga S, Jaffe ES, Steinberg AD, Cossman J. "Clonality of angioimmunoblastic lymphadenopathy and implications for its evolution to malignant lymphoma." '''J Clin Invest'''. 1987 Feb;79(2):637-42. PMID: 3805286</ref> Similarly, [[EBV]]-related sequences can be detected most cases, usually in [[B-cells]] but occasionally in [[T-cells]].<ref name="wei1"/><ref name="ana1"/>.  [[Trisomy]] 3, trisomy 5, and +X are the most frequent chromosomal abnormalities found in cases.<ref name="kane1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=3261178&query_hl=40&itool=pubmed_ExternalLink]
Feller AC, Griesser H, Schilling CV, Wacker HH, Dallenbach F, Bartels H, Kuse R, Mak TW, Lennert K. "Clonal gene rearrangement patterns correlate with immunophenotype and clinical parameters in patients with angioimmunoblastic lymphadenopathy." '''Am J Pathol'''. 1988 Dec;133(3):549-56. PMID: 2849301</ref>
Kaneko Y, Maseki N, Sakurai M, Takayama S, Nanba K, Kikuchi M, Frizzera G. "Characteristic karyotypic pattern in T-cell lymphoproliferative disorders with reactive "angioimmunoblastic lymphadenopathy with dysproteinemia-type" features." '''Blood'''. 1988 Aug;72(2):413-21. PMID: 3261178</ref><ref name="sch1">
* [[immunoglobin]] [[gene]] rearrangements are detected in 10% of the cases.<ref name="lip1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=3805286&query_hl=36&itool=pubmed_ExternalLink]
[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=7919378&query_hl=42&itool=pubmed_ExternalLink]
Lipford EH, Smith HR, Pittaluga S, Jaffe ES, Steinberg AD, Cossman J. "Clonality of angioimmunoblastic lymphadenopathy and implications for its evolution to malignant lymphoma." '''J Clin Invest'''. 1987 Feb;79(2):637-42. PMID: 3805286</ref>  
* The [[Epstein–Barr virus|Epstein–Barr virus (EBV)]] is observed in the majority of cases, where the [[virus]] has been found in both reactive [[B cell|B-cells]] (that comprise part of the [[Polymorphism (biology)|polymorphous]] infiltrate) and the [[Cancer|neoplastic]] [[T cell|T-cells]].<ref name="wei1">{{cite journal |author=Weiss LM, Jaffe ES, Liu XF, Chen YY, Shibata D, Medeiros LJ |title=Detection and localization of Epstein-Barr viral genomes in angioimmunoblastic lymphadenopathy and angioimmunoblastic lymphadenopathy-like lymphoma |journal=Blood |volume=79 |issue=7 |pages=1789–95 |date=April 1992 |pmid=1373088 |url=http://www.bloodjournal.org/content/79/7/1789.full}}
</ref><ref name="who1">{{cite book |last=Swerdlow |first=S.H. |last2=Campo |first2=E. |last3=Harris |first3=N.L. |last4=Jaffe |first4=E.S. |last5=Pileri |first5=S.A. |last6=Stein |first6=H. |last7=Thiele |first7=J. |last8=Vardiman |first8=J.W |chapter=11 Mature T- and NK-cell neoplasms: Angioimmunoblastic T-cell lymphoma |title=WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues |edition=4th |series=IARC WHO Classification of Tumours |volume=2 |publisher=IARC |year=2008 |isbn=9283224310 |url=http://apps.who.int/bookorders/anglais/detart1.jsp?codlan=1&codcol=70&codcch=4002&content=1}}</ref><ref name="ana1">{{cite journal |author=Anagnostopoulos I, Hummel M, Finn T |title=Heterogeneous Epstein-Barr virus infection patterns in peripheral T-cell lymphoma of angioimmunoblastic lymphadenopathy type |journal=Blood |volume=80 |issue=7 |pages=1804–12 |date=October 1992 |pmid=1327284 |display-authors=etal |url=http://www.bloodjournal.org/content/80/7/1804.full}}
</ref>  
* [[Aneuploidy|Trisomy]] 3, [[Aneuploidy|trisomy]] 5, and +X are the most frequent [[Chromosome|chromosomal]] abnormalities found in cases.<ref name="kane1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=3261178&query_hl=40&itool=pubmed_ExternalLink]
Kaneko Y, Maseki N, Sakurai M, Takayama S, Nanba K, Kikuchi M, Frizzera G. "Characteristic karyotypic pattern in T-cell lymphoproliferative disorders with reactive "angioimmunoblastic lymphadenopathy with dysproteinemia-type" features." '''Blood'''. 1988 Aug;72(2):413-21. PMID: 3261178</ref><ref name="sch1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=7919378&query_hl=42&itool=pubmed_ExternalLink]
Schlegelberger B, Zhang Y, Weber-Matthiesen K, Grote W. "Detection of aberrant clones in nearly all cases of angioimmunoblastic lymphadenopathy with dysproteinemia-type T-cell lymphoma by combined interphase and metaphase cytogenetics." '''Blood'''. 1994 Oct 15;84(8):2640-8. PMID: 7919378</ref>
Schlegelberger B, Zhang Y, Weber-Matthiesen K, Grote W. "Detection of aberrant clones in nearly all cases of angioimmunoblastic lymphadenopathy with dysproteinemia-type T-cell lymphoma by combined interphase and metaphase cytogenetics." '''Blood'''. 1994 Oct 15;84(8):2640-8. PMID: 7919378</ref>


===Gross Pathology===
==Gross Pathology==
The normal architecture of a lymph node is partially effaced by a polymorphous infiltrate and residual follicles are commonly seen. The polymorphous infiltrate consists of lymphocytes of moderate size with pale/clear cytoplasm and smaller reactive [[lymphocytes]], [[eosinophils]], [[histiocytes]], [[plasma cells]], and [[follicular dendritic cells]]. In addition, blast-like [[B-cells]] are occasionally seen.  A classic morphological finding is the aborization and proliferation of [[high endothelial venules]].<ref name="who1"/> Hyperplastic [[germinal centers]] and [[Reed-Sternberg cells]] can also be seen.<ref name="quin1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10524524&query_hl=30&itool=pubmed_ExternalLink]
A classic [[Morphology|morphological]] finding is the aborization and proliferation of [[high endothelial venules]].<ref name="who1" />
Quintanilla-Martinez L, Fend F, Moguel LR, Spilove L, Beaty MW, Kingma DW, Raffeld M, Jaffe ES. "Peripheral T-cell lymphoma with Reed-Sternberg-like cells of B-cell phenotype and genotype associated with Epstein-Barr virus infection." '''Am J Surg Pathol'''. 1999 Oct;23(10):1233-40. PMID: 10524524</ref><ref name="ree1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=9630171&query_hl=32&itool=pubmed_ExternalLink]
==Microscopic Pathology==
* Angioimmunoblastic T-cell lymphoma typically has the phenotype of a mixture of [[CD4]]+ and [[CD8]]+ [[T-cells]], with a [[CD4]]:[[CD8]] ratio greater than unity. 
* Polyclonal [[plasma cells]], [[lymphocytes]], [[eosinophils]], [[histiocytes]], [[blast]]-like [[B-cells]], and [[CD21]]+ [[follicular dendritic cells]] are also seen.<ref name="who1" />
* Due to the systemic nature of this disease, [[Cancer|neoplastic]] [[Cell (biology)|cells]] can be found in [[lymph nodes]], [[liver]], [[spleen]], [[skin]], and [[bone marrow]].
* [[Hyperplasia|Hyperplastic]] [[germinal centers]] and [[Reed-Sternberg cells]] can also be seen.<ref name="quin1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10524524&query_hl=30&itool=pubmed_ExternalLink] Quintanilla-Martinez L, Fend F, Moguel LR, Spilove L, Beaty MW, Kingma DW, Raffeld M, Jaffe ES. "Peripheral T-cell lymphoma with Reed-Sternberg-like cells of B-cell phenotype and genotype associated with Epstein-Barr virus infection." '''Am J Surg Pathol'''. 1999 Oct;23(10):1233-40. PMID: 10524524</ref><ref name="ree1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=9630171&query_hl=32&itool=pubmed_ExternalLink]
Ree HJ, Kadin ME, Kikuchi M, Ko YH, Go JH, Suzumiya J, Kim DS. "Angioimmunoblastic lymphoma (AILD-type T-cell lymphoma) with hyperplastic germinal centers." '''Am J Surg Pathol'''. 1998 Jun;22(6):643-55. PMID: 9630171</ref>
Ree HJ, Kadin ME, Kikuchi M, Ko YH, Go JH, Suzumiya J, Kim DS. "Angioimmunoblastic lymphoma (AILD-type T-cell lymphoma) with hyperplastic germinal centers." '''Am J Surg Pathol'''. 1998 Jun;22(6):643-55. PMID: 9630171</ref>


===Microscopic Pathology===
AILT typically has the phenotype of a mixture of [[CD4]]+ and [[CD8]]+ [[T-cells]], with a CD4:CD8 ratio greater than unity.  Polyclonal [[plasma cells]] and [[CD21]]+ [[follicular dendritic cells]] are also seen.<ref name="who1"/>  Due to the systemic nature of this disease, neoplastic cells can be found in [[lymph nodes]], [[liver]], [[spleen]], [[skin]], and [[bone marrow]].
===Gallery===
===Gallery===
<gallery widths=200px>
<gallery widths="200px">
Image:Angioimmunoblastic T-cell lymphoma Biopsy 1.jpg|A kidney biopsy showing effacement of the renal structure by diffuse leukocytic infiltrate, represented mostly by elongated cells with marked artifactual changes.<ref name = casereport>Angioimmunoblastic T-cell lymphoma presenting as giant kidneys: a case report
Image:Angioimmunoblastic T-cell lymphoma Biopsy 1.jpg|A kidney biopsy showing effacement of the renal structure by diffuse leukocytic infiltrate, represented mostly by elongated cells with marked artifactual changes.<ref name = casereport>Angioimmunoblastic T-cell lymphoma presenting as giant kidneys: a case report
http://www.jmedicalcasereports.com/content/3/1/9258 Accessed on November,25 2015</ref>
http://www.jmedicalcasereports.com/content/3/1/9258 Accessed on November,25 2015</ref>
Line 25: Line 32:
Image:Angioimmunoblastic T-cell lymphoma Biopsy 3.jpg|The paracortical area was infiltrated by cells that were positive for CD3 and CD4.<ref name = casereport>Angioimmunoblastic T-cell lymphoma presenting as giant kidneys: a case report http://www.jmedicalcasereports.com/content/3/1/9258 Accessed on November,25 2015</ref>
Image:Angioimmunoblastic T-cell lymphoma Biopsy 3.jpg|The paracortical area was infiltrated by cells that were positive for CD3 and CD4.<ref name = casereport>Angioimmunoblastic T-cell lymphoma presenting as giant kidneys: a case report http://www.jmedicalcasereports.com/content/3/1/9258 Accessed on November,25 2015</ref>
</gallery>
</gallery>


==References==
==References==
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Latest revision as of 19:53, 27 March 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [7]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [8] Sowminya Arikapudi, M.B,B.S. [9]

Overview

Clonal T-cell receptor and immunoglobulin gene rearrangements are involved in the pathogenesis of angioimmunoblastic T-cell lymphoma. On gross pathology, aborization and proliferation of high endothelial venules are characteristic findings of angioimmunoblastic T-cell lymphoma. On microscopic histopathological analysis, CD4+ T-cells, CD8+ T-cells, and polyclonal plasma cells are characteristic findings of angioimmunoblastic T-cell lymphoma.

Genetics

Gross Pathology

A classic morphological finding is the aborization and proliferation of high endothelial venules.[4]

Microscopic Pathology

Gallery

  • A kidney biopsy showing effacement of the renal structure by diffuse leukocytic infiltrate, represented mostly by elongated cells with marked artifactual changes.<ref name = casereport>Angioimmunoblastic T-cell lymphoma presenting as giant kidneys: a case report

    A kidney biopsy showing effacement of the renal structure by diffuse leukocytic infiltrate, represented mostly by elongated cells with marked artifactual changes.<ref name = casereport>Angioimmunoblastic T-cell lymphoma presenting as giant kidneys: a case report

  • A biopsy of an inguinal lymph node was remarkable for obliteration of the node architecture.[10]

    A biopsy of an inguinal lymph node was remarkable for obliteration of the node architecture.[10]

  • The paracortical area was infiltrated by cells that were positive for CD3 and CD4.[10]

    The paracortical area was infiltrated by cells that were positive for CD3 and CD4.[10]

References

  1. ↑ [1] Feller AC, Griesser H, Schilling CV, Wacker HH, Dallenbach F, Bartels H, Kuse R, Mak TW, Lennert K. "Clonal gene rearrangement patterns correlate with immunophenotype and clinical parameters in patients with angioimmunoblastic lymphadenopathy." Am J Pathol. 1988 Dec;133(3):549-56. PMID: 2849301
  2. ↑ [2] Lipford EH, Smith HR, Pittaluga S, Jaffe ES, Steinberg AD, Cossman J. "Clonality of angioimmunoblastic lymphadenopathy and implications for its evolution to malignant lymphoma." J Clin Invest. 1987 Feb;79(2):637-42. PMID: 3805286
  3. ↑ Weiss LM, Jaffe ES, Liu XF, Chen YY, Shibata D, Medeiros LJ (April 1992). "Detection and localization of Epstein-Barr viral genomes in angioimmunoblastic lymphadenopathy and angioimmunoblastic lymphadenopathy-like lymphoma". Blood. 79 (7): 1789–95. PMID 1373088.
  4. ↑ 4.0 4.1 4.2 Swerdlow, S.H.; Campo, E.; Harris, N.L.; Jaffe, E.S.; Pileri, S.A.; Stein, H.; Thiele, J.; Vardiman, J.W (2008). "11 Mature T- and NK-cell neoplasms: Angioimmunoblastic T-cell lymphoma". WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. IARC WHO Classification of Tumours. 2 (4th ed.). IARC. ISBN 9283224310.
  5. ↑ Anagnostopoulos I, Hummel M, Finn T; et al. (October 1992). "Heterogeneous Epstein-Barr virus infection patterns in peripheral T-cell lymphoma of angioimmunoblastic lymphadenopathy type". Blood. 80 (7): 1804–12. PMID 1327284.
  6. ↑ [3] Kaneko Y, Maseki N, Sakurai M, Takayama S, Nanba K, Kikuchi M, Frizzera G. "Characteristic karyotypic pattern in T-cell lymphoproliferative disorders with reactive "angioimmunoblastic lymphadenopathy with dysproteinemia-type" features." Blood. 1988 Aug;72(2):413-21. PMID: 3261178
  7. ↑ [4] Schlegelberger B, Zhang Y, Weber-Matthiesen K, Grote W. "Detection of aberrant clones in nearly all cases of angioimmunoblastic lymphadenopathy with dysproteinemia-type T-cell lymphoma by combined interphase and metaphase cytogenetics." Blood. 1994 Oct 15;84(8):2640-8. PMID: 7919378
  8. ↑ [5] Quintanilla-Martinez L, Fend F, Moguel LR, Spilove L, Beaty MW, Kingma DW, Raffeld M, Jaffe ES. "Peripheral T-cell lymphoma with Reed-Sternberg-like cells of B-cell phenotype and genotype associated with Epstein-Barr virus infection." Am J Surg Pathol. 1999 Oct;23(10):1233-40. PMID: 10524524
  9. ↑ [6] Ree HJ, Kadin ME, Kikuchi M, Ko YH, Go JH, Suzumiya J, Kim DS. "Angioimmunoblastic lymphoma (AILD-type T-cell lymphoma) with hyperplastic germinal centers." Am J Surg Pathol. 1998 Jun;22(6):643-55. PMID: 9630171
  10. ↑ 10.0 10.1 Angioimmunoblastic T-cell lymphoma presenting as giant kidneys: a case report http://www.jmedicalcasereports.com/content/3/1/9258 Accessed on November,25 2015


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