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*Lymphogranulomatosis X
*Lymphogranulomatosis X
==Epidemiology / Prevalence==
<ref>{{Cite journal|last=L|first=Rauramo|last2=K|first2=Lagerspetz|last3=P|first3=Engblom|last4=R|first4=Punnonen|date=1975|title=The effect of castration and peroral estrogen therapy on some psychological functions|url=https://pubmed.ncbi.nlm.nih.gov/1234567/|language=en|pmid=1234567}}</ref>
*Occurs in middle-aged and elderly individuals<ref name=":0">{{Cite journal|last=L|first=de Leval|last2=C|first2=Gisselbrecht|last3=P|first3=Gaulard|date=2010|title=Advances in the understanding and management of angioimmunoblastic T-cell lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/19961485/|language=en|pmid=19961485}}</ref>
*Males >> Females<ref name=":0" />
*One of the most common specific subtypes of PTCL<ref>{{Cite journal|last=M|first=Vences|last2=Jm|first2=Guayasamin|last3=A|first3=Miralles|last4=I|first4=De la Riva|date=2013|title=To name or not to name: Criteria to promote economy of change in Linnaean classification schemes|url=https://pubmed.ncbi.nlm.nih.gov/26042291/|language=en|pmid=26042291}}</ref><ref>{{Cite journal|last=T|first=Rüdiger|last2=Dd|first2=Weisenburger|last3=Jr|first3=Anderson|last4=Jo|first4=Armitage|last5=J|first5=Diebold|last6=Ka|first6=MacLennan|last7=Bn|first7=Nathwani|last8=F|first8=Ullrich|last9=Hk|first9=Müller-Hermelink|date=2002|title=Peripheral T-cell lymphoma (excluding anaplastic large-cell lymphoma): results from the Non-Hodgkin's Lymphoma Classification Project|url=https://pubmed.ncbi.nlm.nih.gov/11863096/|language=en|pmid=11863096}}</ref><ref>{{Cite journal|last=J|first=Vose|last2=J|first2=Armitage|last3=D|first3=Weisenburger|date=2008|title=International peripheral T-cell and natural killer/T-cell lymphoma study: pathology findings and clinical outcomes|url=https://pubmed.ncbi.nlm.nih.gov/18626005/|language=en|pmid=18626005}}</ref>
**15-30% of non-cutaneous T-cell lymphomas
**1-2% of all non-Hodgkin lymphomas
==Clinical Features<small><ref>{{Cite journal|last=A|first=Dogan|last2=Ad|first2=Attygalle|last3=C|first3=Kyriakou|date=2003|title=Angioimmunoblastic T-cell lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/12780782/|language=en|pmid=12780782}}</ref><ref>{{Cite journal|last=F|first=Lachenal|last2=F|first2=Berger|last3=H|first3=Ghesquières|last4=P|first4=Biron|last5=A|first5=Hot|last6=E|first6=Callet-Bauchu|last7=C|first7=Chassagne|last8=B|first8=Coiffier|last9=I|first9=Durieu|date=2007|title=Angioimmunoblastic T-cell lymphoma: clinical and laboratory features at diagnosis in 77 patients|url=https://pubmed.ncbi.nlm.nih.gov/17873758/|language=en|pmid=17873758}}</ref><ref>{{Cite journal|last=N|first=Mourad|last2=N|first2=Mounier|last3=J|first3=Brière|last4=E|first4=Raffoux|last5=A|first5=Delmer|last6=A|first6=Feller|last7=Cj|first7=Meijer|last8=Jf|first8=Emile|last9=R|first9=Bouabdallah|date=2008|title=Clinical, biologic, and pathologic features in 157 patients with angioimmunoblastic T-cell lymphoma treated within the Groupe d'Etude des Lymphomes de l'Adulte (GELA) trials|url=https://pubmed.ncbi.nlm.nih.gov/18292286/|language=en|doi=10.1182/blood-2007-08-105759|pmc=PMC2343588|pmid=18292286}}</ref><ref>{{Cite journal|last=W|first=Siegert|last2=C|first2=Nerl|last3=A|first3=Agthe|last4=M|first4=Engelhard|last5=G|first5=Brittinger|last6=M|first6=Tiemann|last7=K|first7=Lennert|last8=D|first8=Huhn|date=1995|title=Angioimmunoblastic lymphadenopathy (AILD)-type T-cell lymphoma: prognostic impact of clinical observations and laboratory findings at presentation. The Kiel Lymphoma Study Group|url=https://pubmed.ncbi.nlm.nih.gov/8664186/|language=en|pmid=8664186}}</ref></small>==
==Clinical Features<small><ref>{{Cite journal|last=A|first=Dogan|last2=Ad|first2=Attygalle|last3=C|first3=Kyriakou|date=2003|title=Angioimmunoblastic T-cell lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/12780782/|language=en|pmid=12780782}}</ref><ref>{{Cite journal|last=F|first=Lachenal|last2=F|first2=Berger|last3=H|first3=Ghesquières|last4=P|first4=Biron|last5=A|first5=Hot|last6=E|first6=Callet-Bauchu|last7=C|first7=Chassagne|last8=B|first8=Coiffier|last9=I|first9=Durieu|date=2007|title=Angioimmunoblastic T-cell lymphoma: clinical and laboratory features at diagnosis in 77 patients|url=https://pubmed.ncbi.nlm.nih.gov/17873758/|language=en|pmid=17873758}}</ref><ref>{{Cite journal|last=N|first=Mourad|last2=N|first2=Mounier|last3=J|first3=Brière|last4=E|first4=Raffoux|last5=A|first5=Delmer|last6=A|first6=Feller|last7=Cj|first7=Meijer|last8=Jf|first8=Emile|last9=R|first9=Bouabdallah|date=2008|title=Clinical, biologic, and pathologic features in 157 patients with angioimmunoblastic T-cell lymphoma treated within the Groupe d'Etude des Lymphomes de l'Adulte (GELA) trials|url=https://pubmed.ncbi.nlm.nih.gov/18292286/|language=en|doi=10.1182/blood-2007-08-105759|pmc=PMC2343588|pmid=18292286}}</ref><ref>{{Cite journal|last=W|first=Siegert|last2=C|first2=Nerl|last3=A|first3=Agthe|last4=M|first4=Engelhard|last5=G|first5=Brittinger|last6=M|first6=Tiemann|last7=K|first7=Lennert|last8=D|first8=Huhn|date=1995|title=Angioimmunoblastic lymphadenopathy (AILD)-type T-cell lymphoma: prognostic impact of clinical observations and laboratory findings at presentation. The Kiel Lymphoma Study Group|url=https://pubmed.ncbi.nlm.nih.gov/8664186/|language=en|pmid=8664186}}</ref></small>==
'''Sign and Symptoms'''
'''Sign and Symptoms'''
==Chromosomal Rearrangements (Gene Fusions)==
==Chromosomal Rearrangements (Gene Fusions)==
{| class="wikitable sortable"
|-
!Chromosomal Rearrangement!!Genes in Fusion (5’ or 3’ Segments)!!Pathogenic Derivative!!Reference
|-
|t(5;9)(q33;q22)||''ITK'' / ''SYK''||der(5); der(9)||<ref>{{Cite journal|last=B|first=Streubel|last2=U|first2=Vinatzer|last3=M|first3=Willheim|last4=M|first4=Raderer|last5=A|first5=Chott|date=2006|title=Novel t(5;9)(q33;q22) fuses ITK to SYK in unspecified peripheral T-cell lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/16341044/|language=en|pmid=16341044}}</ref><ref name=":4">{{Cite journal|last=M|first=Wang|last2=S|first2=Zhang|last3=Ss|first3=Chuang|last4=M|first4=Ashton-Key|last5=E|first5=Ochoa|last6=N|first6=Bolli|last7=G|first7=Vassiliou|last8=Z|first8=Gao|last9=Mq|first9=Du|date=2017|title=Angioimmunoblastic T cell lymphoma: novel molecular insights by mutation profiling|url=https://pubmed.ncbi.nlm.nih.gov/28148900/|language=en|doi=10.18632/oncotarget.14846|pmc=PMC5392284|pmid=28148900}}</ref>
|-
|t(7;14)(q35;q32.1)
|''TRB/TCL1A''
|der(7); der(14)
|<ref>{{Cite journal|last=Mf|first=Cosimi|last2=I|first2=Casagranda|last3=G|first3=Ghiazza|last4=G|first4=Rossi|last5=P|first5=Galvani|date=1990|title=Rearrangements on chromosomes 7 and 14 with breakpoints at 7q35 and 14q11 in angioimmunoblastic lymphadenopathy and IBL-like T-cell lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/2284141/|language=en|pmid=2284141}}</ref>
|-
|t(14;14)(q11;q32.1) / inv(14)(q11q32.1)
|''TRA-TRD/TCL1A''
|der(14)
|<ref>{{Cite journal|last=B|first=Schlegelberger|last2=A|first2=Feller|last3=A|first3=Himmler|last4=W|first4=Grote|date=1990|title=Inv(14)(q11q32) in one of four different clones in a case of angioimmunoblastic lymphadenopathy|url=https://pubmed.ncbi.nlm.nih.gov/2293883/|language=en|pmid=2293883}}</ref><ref>{{Cite journal|last=E|first=Leich|last2=E|first2=Haralambieva|last3=A|first3=Zettl|last4=A|first4=Chott|last5=T|first5=Rüdiger|last6=S|first6=Höller|last7=Hk|first7=Müller-Hermelink|last8=G|first8=Ott|last9=A|first9=Rosenwald|date=2007|title=Tissue microarray-based screening for chromosomal breakpoints affecting the T-cell receptor gene loci in mature T-cell lymphomas|url=https://pubmed.ncbi.nlm.nih.gov/17582237/|language=en|pmid=17582237}}</ref>
|-
|chr(2)(q33.2)
|''CTLA4/CD28''
|der(2)
|<ref>{{Cite journal|last=Hy|first=Yoo|last2=P|first2=Kim|last3=Ws|first3=Kim|last4=Sh|first4=Lee|last5=S|first5=Kim|last6=Sy|first6=Kang|last7=Hy|first7=Jang|last8=Je|first8=Lee|last9=J|first9=Kim|date=2016|title=Frequent CTLA4-CD28 gene fusion in diverse types of T-cell lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/26819049/|language=en|doi=10.3324/haematol.2015.139253|pmc=PMC5013939|pmid=26819049}}</ref>
|}
==Characteristic Chromosomal Aberrations / Patterns==
==Characteristic Chromosomal Aberrations / Patterns==
**''TET2'' (50-80%)
**''TET2'' (50-80%)
**''DNMT3A'' (20-30%)
**''DNMT3A'' (20-30%)
==Genes and Main Pathways Involved==
{| class="wikitable"
|+
!Molecular Features
!Pathway
!Pathophysiologic Outcome
|-
|''FYN'', ''PLCG1'', and ''CD28'' mutations
|T-cell receptor signaling pathway<ref name=":4" /><ref name=":6" /><ref name=":3" /><ref name=":13" />
|Increased proliferation and survival
|-
|''IDH2'', ''TET2'', and ''DNMT3A'' mutations
|Histone modification and chromatin remodeling<ref name=":4" /><ref name=":5" /><ref name=":11" /><ref name=":12" />
|Abnormal gene expression program
|}
*
==Diagnostic Testing Methods==
==Diagnostic Testing Methods==
*''IDH2'' R172 mutations are specific to AITL
*''IDH2'' R172 mutations are specific to AITL
*T-Cell receptor and immunoglobulin genes rearrangement detection by karyotyping and FISH analysis
*T-Cell receptor and immunoglobulin genes rearrangement detection by karyotyping and FISH analysis
==Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications)==
*Overall prognosis is poor<ref name=":9">{{Cite journal|last=N|first=Mourad|last2=N|first2=Mounier|last3=J|first3=Brière|last4=E|first4=Raffoux|last5=A|first5=Delmer|last6=A|first6=Feller|last7=Cj|first7=Meijer|last8=Jf|first8=Emile|last9=R|first9=Bouabdallah|date=2008|title=Clinical, biologic, and pathologic features in 157 patients with angioimmunoblastic T-cell lymphoma treated within the Groupe d'Etude des Lymphomes de l'Adulte (GELA) trials|url=https://pubmed.ncbi.nlm.nih.gov/18292286/|language=en|doi=10.1182/blood-2007-08-105759|pmc=PMC2343588|pmid=18292286}}</ref><ref name=":10">{{Cite journal|last=J|first=Vose|last2=J|first2=Armitage|last3=D|first3=Weisenburger|date=2008|title=International peripheral T-cell and natural killer/T-cell lymphoma study: pathology findings and clinical outcomes|url=https://pubmed.ncbi.nlm.nih.gov/18626005/|language=en|pmid=18626005}}</ref>
*Median survival < 3 years, even with aggressive treatment<ref name=":9" /><ref name=":10" />
*Male sex, mediastinal lymphadenopathy, and anemia adversely affect the survival<ref name=":9" />
'''Suggested Treatment Regimens based on NCCN Guideline Version 1.2020 (TCEL-B 3 of 5)'''
'''Suggested Treatment Regimens based on NCCN Guideline Version 1.2020 (TCEL-B 3 of 5)'''
[[Angioimmunoblastic T-cell Lymphoma and Other Nodal Lymphomas of T Follicular Helper Cell Origin]]
[[Angioimmunoblastic T-cell Lymphoma and Other Nodal Lymphomas of T Follicular Helper Cell Origin]]
==References==
==References==
(use "Cite" icon at top of page)
(use "Cite" icon at top of page)
<references />
<references />
===EXAMPLE Book===
#Arber DA, et al., (2017). Acute myeloid leukaemia with recurrent genetic abnormalities, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. IARC Press: Lyon, France, p129-171.
==Notes==
==Notes==
<nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page. If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage). Additional global feedback or concerns are also welcome.
<nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page. If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage). Additional global feedback or concerns are also welcome.