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Difference between revisions of "Enteropathy-associated T-cell lymphoma"
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Enteropathy-associated T-cell lymphoma (view source)
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| Caption = Enteropathy-associated T cell lymphoma. [[H&E stain]]. | | Caption = Enteropathy-associated T cell lymphoma. [[H&E stain]]. | ||
| Synonyms = enteropathy-type T-cell lymphoma | | Synonyms = enteropathy-type T-cell lymphoma | ||
| Micro = large | | Micro = large or medium-sized lymphoid cells in the mucosa and submucosa | ||
| Subtypes = | | Subtypes = Historical: pleomorphic anaplastic (type I), monomorphic (type II) | ||
| LMDDx = | | LMDDx = other lymphomas | ||
| Stains = | | Stains = | ||
| IHC = CD3 +ve, CD5 -ve, CD8 -ve/+ve, CD56 -ve/+ve, CD30 +ve | | IHC = CD3 +ve, CD5 -ve, CD8 -ve/+ve, CD56 -ve/+ve, CD30 +ve | ||
| Line 25: | Line 25: | ||
| Rads = | | Rads = | ||
| Endoscopy = | | Endoscopy = | ||
| Prognosis = | | Prognosis = poor | ||
| Other = | | Other = | ||
| ClinDDx = | | ClinDDx = dependent on presentation - other causes of [[SBO]], e.g. [[duodenal adenocarcinoma]] | ||
| Tx = | | Tx = | ||
}} | }} | ||
| Line 35: | Line 35: | ||
==General== | ==General== | ||
*T cell lymphoma due to [[celiac sprue]]. | *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== | ==Gross== | ||
| Line 53: | Line 65: | ||
DDx: | DDx: | ||
*Other types of lymphoma | *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=== | ===Image=== | ||
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Image:Enteropathy-associated_T_cell_lymphoma_-_low_mag.jpg | EATL. (WC) | Image:Enteropathy-associated_T_cell_lymphoma_-_low_mag.jpg | EATL. (WC) | ||
</gallery> | </gallery> | ||
==IHC== | ==IHC== | ||
Features - type 1:<ref name=pmid21323966/> | 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]] | *[[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> | 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> | ||
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*CD5 -ve. | *CD5 -ve. | ||
*CD8 -ve/+ve. | *CD8 -ve/+ve. | ||
*CD56 | *CD56 +ve. | ||
*CD30 +ve. | *CD30 +ve. | ||