View source for HAEM4:Intestinal T-cell Lymphoma, Not Otherwise Specified (NOS)

{{DISPLAYTITLE:Intestinal T-cell Lymphoma, Not Otherwise Specified (NOS)}}


<blockquote class='blockedit'>{{Box-round|title=PREVIOUS EDITION|This page from the 4th edition of Haematolymphoid Tumours is being updated. See 5th edition [[HAEM5:Table_of_Contents|Table of Contents]].
}}</blockquote>
==Primary Author(s)*==

Derick Okwan MD, PhD (Stanford Medicine, CA) 

Sumire Kitahara MD (Cedars-Sinai, CA) 

__TOC__

==Cancer Category/Type==

[[HAEM4:Mature T- and NK-cell Neoplasms]]

==Cancer Sub-Classification / Subtype==

[[HAEM4:Intestinal T-cell Lymphoma]]

==Definition / Description of Disease==

*Category that is assigned T-cell lymphoma entities that do not meet diagnostic criteria for [[HAEM5:Enteropathy-associated T-cell lymphoma]] or [[HAEM5:Monomorphic epitheliotropic intestinal T-cell lymphoma]]
*Typically assigned in the setting of inadequate biopsy, equivocal assessment of mucosal surface, incomplete immunophenotype, or incomplete clinical history where celiac disease is uninvolved
*Not a distinct clinical entity

==Synonyms / Terminology==

*N/A

==Epidemiology / Prevalence==

*N/A

==Clinical Features==

*Aggressive disease with typically widespread involvement<ref name=":0">{{Cite journal|last=Attygalle|first=Ayoma D|last2=Cabeçadas|first2=José|last3=Gaulard|first3=Philippe|last4=Jaffe|first4=Elaine S|last5=de Jong|first5=Daphne|last6=Ko|first6=Young Hyeh|last7=Said|first7=Jonathan|last8=Klapper|first8=Wolfram|date=2014|title=Peripheral T-cell and NK-cell lymphomas and their mimics; taking a step forward - report on the lymphoma workshop of the XVIth meeting of the European Association for Haematopathology and the Society for Hematopathology|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6364972/|journal=Histopathology|volume=64|issue=2|pages=171–199|doi=10.1111/his.12251|issn=0309-0167|pmc=6364972|pmid=24128129}}</ref>
*Not associated with celiac disease<ref name=":0" />
*Weight loss, anorexia, fatigue, possible intestinal obstruction/perforation<ref name=":0" />
*May not respond to standard therapy indicated for enteropathy-associated T-cell lymphoma or monomorphic epitheliotropic intestinal T-cell lymphoma<ref name=":0" />

==Sites of Involvement==

*Intestinal tract (predominantly colon)

==Morphologic Features==

*Heterogeneous, variable-sized lymphocytic infiltrate
*Shares features compatible with both enteropathy-associated T-cell lymphoma and monomorphic epitheliotropic intestinal T-cell lymphoma

==Immunophenotype==

*N/A

==Chromosomal Rearrangements (Gene Fusions)==

*No unique disease-specific genetic features

==Characteristic Chromosomal Aberrations / Patterns==

*No unique disease-specific chromosomal pattern

==Genomic Gain/Loss/LOH==

*N/A

==Gene Mutations (SNV/INDEL)==

*N/A

===Other Mutations===

*N/A

==Epigenomics (Methylation)==

*Not described

==Genes and Main Pathways Involved==

*N/A

==Diagnostic Testing Methods==

*Must rule out definitive diagnosis of enteropathy-associated T-cell lymphoma and monomorphic epitheliotropic intestinal T-cell lymphoma

==Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications)==

*Aggressive course
*Must rule out all definitive diagnoses, including enteropathy-associated T-cell lymphoma and monomorphic epitheliotropic intestinal T-cell lymphoma and indolent T-cell lymphoproliferative disorder of the gastrointestinal tract

==Familial Forms==

*None

==Other Information==

*See [[HAEM4:Intestinal T-cell Lymphoma]]

==Links==

[[HAEM4:Intestinal T-cell Lymphoma]]

<br />

==References==

<references />

#

==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.
[[Category:HAEM4]] [[Category:DISEASE]]