Difference between revisions of "Enteropathy-associated T-cell lymphoma"

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Enteropathy-associated T-cell lymphoma (view source)

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#redirect [[Lymphoma#Enteropathy-associated_T-cell_lymphoma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Enteropathy-associated_T_cell_lymphoma_-_low_mag.jpg
| Width      =
| Caption    = Enteropathy-associated T cell lymphoma. [[H&E stain]].
| Synonyms  = enteropathy-type T-cell lymphoma
| Micro      = large or medium-sized lymphoid cells in the mucosa and submucosa
| Subtypes  = Historical: pleomorphic anaplastic (type I), monomorphic (type II)
| LMDDx      = other lymphomas
| Stains    =
| IHC        = CD3 +ve, CD5 -ve, CD8 -ve/+ve, CD56 -ve/+ve, CD30 +ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[duodenum]]
| Assdx      = [[celiac disease]]
| Syndromes  =
| Clinicalhx = history of celiac disease
| Signs      =
| Symptoms  =
| Prevalence = uncommon
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = poor
| Other      =
| ClinDDx    = dependent on presentation - other causes of [[SBO]], e.g. [[duodenal adenocarcinoma]]
| Tx        =
}}
'''Enteropathy-associated T-cell lymphoma''', abbreviated '''EATL''', is an uncommon type of [[lymphoma]] associated with [[celiac disease]].
 
It is also known as '''enteropathy-type T-cell lymphoma''', abbreviated '''ETTL'''.
 
==General==
*T cell lymphoma classically due to [[celiac sprue]].
*Historically subdivided into type I and type II.<ref name=pmid21566094>{{cite journal |author=Delabie J, et al |title=Enteropathy-associated T-cell lymphoma: clinical and histological findings from the International Peripheral T-Cell Lymphoma Project|journal=Blood |volume=118|issue=148|pages= 148|year=2011|month=July}}</ref>
**''Pleomorphic anaplastic'' (type I) associated with celiac sprue.<ref name=pmid17511112/> - this is now referred to as EATL since the 2017 revision of the WHO blue book 4th edition.
**''Monomorphic'' (type II) not associated with celiac sprue - this is separate entity known as [[monomorphic epitheliotropic intestinal T-cell lymphoma]] (MEITL); it is no longer referred to as EATL as it is not associated with an enteropathy.
*Poor prognosis.<ref name=pmid23313469>{{Cite journal  | last1 = Malamut | first1 = G. | last2 = Chandesris | first2 = O. | last3 = Verkarre | first3 = V. | last4 = Meresse | first4 = B. | last5 = Callens | first5 = C. | last6 = Macintyre | first6 = E. | last7 = Bouhnik | first7 = Y. | last8 = Gornet | first8 = JM. | last9 = Allez | first9 = M. | title = Enteropathy associated T cell lymphoma in celiac disease: a large retrospective study. | journal = Dig Liver Dis | volume = 45 | issue = 5 | pages = 377-84 | month = May | year = 2013 | doi = 10.1016/j.dld.2012.12.001 | PMID = 23313469 }}</ref>
**Five-year survival as low as 20%.<ref name=pmid24876396>{{Cite journal  | last1 = Pun | first1 = AH. | last2 = Kasmeridis | first2 = H. | last3 = Rieger | first3 = N. | last4 = Loganathan | first4 = A. | title = Enteropathy associated T-cell lymphoma presenting with multiple episodes of small bowel haemorrhage and perforation. | journal = J Surg Case Rep | volume = 2014 | issue = 3 | pages =  | month =  | year = 2014 | doi = 10.1093/jscr/rju013 | PMID = 24876396 }}</ref>
 
Presentations - may include:
*[[Small bowel obstruction]] (SBO) or perforation.<ref name=pmid23555174>{{Cite journal  | last1 = Kim | first1 = JB. | last2 = Kim | first2 = SH. | last3 = Cho | first3 = YK. | last4 = Ahn | first4 = SB. | last5 = Jo | first5 = YJ. | last6 = Park | first6 = YS. | last7 = Lee | first7 = JH. | last8 = Kim | first8 = DH. | last9 = Lee | first9 = H. | title = A case of colon perforation due to enteropathy-associated T-cell lymphoma. | journal = World J Gastroenterol | volume = 19 | issue = 11 | pages = 1841-4 | month = Mar | year = 2013 | doi = 10.3748/wjg.v19.i11.1841 | PMID = 23555174 }}</ref>
*GI hemorrhage.<ref name=pmid24876396/>
 
Treatment:
*Chemotherapy.<ref>{{Cite journal  | last1 = Cairoli | first1 = A. | last2 = Ketterer | first2 = N. | last3 = Barelli | first3 = S. | last4 = Duchosal | first4 = MA. | title = High-dose therapy and autologous hematopoietic stem cell transplant in T-cell lymphoma: a single center experience. | journal = Leuk Lymphoma | volume = 55 | issue = 8 | pages = 1827-31 | month = Aug | year = 2014 | doi = 10.3109/10428194.2013.852666 | PMID = 24138331 }}</ref>
*Autologous stem cell transplantation.<ref name=pmid23361910>{{Cite journal  | last1 = Jantunen | first1 = E. | last2 = Boumendil | first2 = A. | last3 = Finel | first3 = H. | last4 = Luan | first4 = JJ. | last5 = Johnson | first5 = P. | last6 = Rambaldi | first6 = A. | last7 = Haynes | first7 = A. | last8 = Duchosal | first8 = MA. | last9 = Bethge | first9 = W. | title = Autologous stem cell transplantation for enteropathy-associated T-cell lymphoma: a retrospective study by the EBMT. | journal = Blood | volume = 121 | issue = 13 | pages = 2529-32 | month = Mar | year = 2013 | doi = 10.1182/blood-2012-11-466839 | PMID = 23361910 }}</ref>
 
==Gross==
Features:<ref name=pmid24959225>{{Cite journal  | last1 = Jiao | first1 = G. | last2 = Zheng | first2 = Z. | last3 = Jiang | first3 = K. | last4 = Zhang | first4 = J. | last5 = Wang | first5 = B. | title = Enteropathy-associated T-cell lymphoma presenting with gastrointestinal tract symptoms: A report of two cases and review of diagnostic challenges and clinicopathological correlation. | journal = Oncol Lett | volume = 8 | issue = 1 | pages = 91-94 | month = Jul | year = 2014 | doi = 10.3892/ol.2014.2105 | PMID = 24959225 }}</ref>
*Typically manifest as ulcers, fissures or plaques. †
 
Notes:
*† B-cell lymphomas are typically raised nodules.<ref name=pmid24959225/>
 
==Microscopic==
Features:<ref name=pmid21323966>{{Cite journal  | last1 = Takeshita | first1 = M. | last2 = Nakamura | first2 = S. | last3 = Kikuma | first3 = K. | last4 = Nakayama | first4 = Y. | last5 = Nimura | first5 = S. | last6 = Yao | first6 = T. | last7 = Urabe | first7 = S. | last8 = Ogawara | first8 = S. | last9 = Yonemasu | first9 = H. | title = Pathological and immunohistological findings and genetic aberrations of intestinal enteropathy-associated T cell lymphoma in Japan. | journal = Histopathology | volume = 58 | issue = 3 | pages = 395-407 | month = Feb | year = 2011 | doi = 10.1111/j.1365-2559.2011.03768.x | PMID = 21323966 }}</ref>
*Abundant large ''or'' medium-sized lymphoid cells in the mucosa and submucosa.
**Large-sized cells ~ 2x RBC diameter = type 1.
**Medium sized cells ~ 1.5x RBC diameter = type 2.
*Intestinal epithelium usually preserved.
 
DDx:
*Other types of lymphoma.
**[[DLBCL]].
**[[Anaplastic large cell lymphoma]].<ref name=pmid24325295>{{Cite journal  | last1 = Kim | first1 = do H. | last2 = Lee | first2 = D. | last3 = Kim | first3 = JW. | last4 = Huh | first4 = J. | last5 = Park | first5 = SH. | last6 = Ha | first6 = HK. | last7 = Suh | first7 = C. | last8 = Yoon | first8 = SM. | last9 = Kim | first9 = KJ. | title = Endoscopic and clinical analysis of primary T-cell lymphoma of the gastrointestinal tract according to pathological subtype. | journal = J Gastroenterol Hepatol | volume = 29 | issue = 5 | pages = 934-43 | month = May | year = 2014 | doi = 10.1111/jgh.12471 | PMID = 24325295 }}</ref>
 
===Image===
<gallery>
Image:Enteropathy-associated_T_cell_lymphoma_-_low_mag.jpg | EATL. (WC)
</gallery>
 
==IHC==
Features - type 1:<ref name=pmid21323966/><ref name=pmid17511112>{{cite journal |authors=Zettl A, deLeeuw R, Haralambieva E, Mueller-Hermelink HK |title=Enteropathy-type T-cell lymphoma |journal=Am. J. Clin. Pathol. |volume=127 |issue=5 |pages=701–6 |date=May 2007 |pmid=17511112 |doi=10.1309/nw2bk1dxb0eqg55h |url=}}</ref>
*[[CD56]] -ve.
 
Features - type 2:<ref name=pmid21323966/><ref name=pmid21921780>{{Cite journal  | last1 = Chan | first1 = JK. | last2 = Chan | first2 = AC. | last3 = Cheuk | first3 = W. | last4 = Wan | first4 = SK. | last5 = Lee | first5 = WK. | last6 = Lui | first6 = YH. | last7 = Chan | first7 = WK. | title = Type II enteropathy-associated T-cell lymphoma: a distinct aggressive lymphoma with frequent γδ T-cell receptor expression. | journal = Am J Surg Pathol | volume = 35 | issue = 10 | pages = 1557-69 | month = Oct | year = 2011 | doi = 10.1097/PAS.0b013e318222dfcd | PMID = 21921780 }}</ref>
*CD3 +ve.
*CD5 -ve.
*CD8 -ve/+ve.
*CD56 +ve.
*CD30 +ve.
 
==See also==
*[[Lymphoma]].
*[[Celiac disease]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Lymphoma]]
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