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

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{{ Infobox diagnosis
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Name      = {{PAGENAME}}
| Image      = Enteropathy-associated_T_cell_lymphoma_-_low_mag.jpg  
| Image      = Enteropathy-associated_T_cell_lymphoma_-_low_mag.jpg
| Width      =
| Width      =
| 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 ''or'' medium-sized lymphoid cells in the mucosa and submucosa
| 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
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| Rads      =
| Rads      =
| Endoscopy  =
| Endoscopy  =
| Prognosis  =
| Prognosis  = poor
| Other      =
| Other      =
| ClinDDx    =
| ClinDDx    = dependent on presentation - other causes of [[SBO]], e.g. [[duodenal adenocarcinoma]]
| Tx        =
| Tx        =
}}
}}
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==General==
==General==
*T cell lymphoma due to [[celiac sprue]].
*T cell lymphoma classically due to [[celiac sprue]].
*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>
*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==
==Microscopic==
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DDx:
DDx:
*Other types of lymphoma, e.g. [[DLBCL]].
*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]] +ve.
*[[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 -ve/+ve.
*CD56 +ve.
*CD30 +ve.
*CD30 +ve.


Latest revision as of 17:18, 25 March 2021

Enteropathy-associated T-cell lymphoma
Diagnosis in short

Enteropathy-associated T cell lymphoma. H&E stain.
Synonyms enteropathy-type T-cell lymphoma
LM large or medium-sized lymphoid cells in the mucosa and submucosa
Subtypes Historical: pleomorphic anaplastic (type I), monomorphic (type II)
LM DDx other lymphomas
IHC CD3 +ve, CD5 -ve, CD8 -ve/+ve, CD56 -ve/+ve, CD30 +ve
Site duodenum
Associated Dx celiac disease
Clinical history history of celiac disease
Prevalence uncommon
Prognosis poor
Clin. DDx dependent on presentation - other causes of SBO, e.g. duodenal adenocarcinoma

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.[1]
    • Pleomorphic anaplastic (type I) associated with celiac sprue.[2] - 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.[3]
    • Five-year survival as low as 20%.[4]

Presentations - may include:

Treatment:

  • Chemotherapy.[6]
  • Autologous stem cell transplantation.[7]

Gross

Features:[8]

  • Typically manifest as ulcers, fissures or plaques. †

Notes:

  • † B-cell lymphomas are typically raised nodules.[8]

Microscopic

Features:[9]

  • 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:

Image

  • EATL. (WC)

IHC

Features - type 1:[9][2]

Features - type 2:[9][11]

  • CD3 +ve.
  • CD5 -ve.
  • CD8 -ve/+ve.
  • CD56 +ve.
  • CD30 +ve.

See also

References

  1. ↑ Delabie J, et al (July 2011). "Enteropathy-associated T-cell lymphoma: clinical and histological findings from the International Peripheral T-Cell Lymphoma Project". Blood 118 (148): 148.
  2. ↑ 2.0 2.1 Zettl A, deLeeuw R, Haralambieva E, Mueller-Hermelink HK (May 2007). "Enteropathy-type T-cell lymphoma". Am. J. Clin. Pathol. 127 (5): 701–6. doi:10.1309/nw2bk1dxb0eqg55h. PMID 17511112.
  3. ↑ Malamut, G.; Chandesris, O.; Verkarre, V.; Meresse, B.; Callens, C.; Macintyre, E.; Bouhnik, Y.; Gornet, JM. et al. (May 2013). "Enteropathy associated T cell lymphoma in celiac disease: a large retrospective study.". Dig Liver Dis 45 (5): 377-84. doi:10.1016/j.dld.2012.12.001. PMID 23313469.
  4. ↑ 4.0 4.1 Pun, AH.; Kasmeridis, H.; Rieger, N.; Loganathan, A. (2014). "Enteropathy associated T-cell lymphoma presenting with multiple episodes of small bowel haemorrhage and perforation.". J Surg Case Rep 2014 (3). doi:10.1093/jscr/rju013. PMID 24876396.
  5. ↑ Kim, JB.; Kim, SH.; Cho, YK.; Ahn, SB.; Jo, YJ.; Park, YS.; Lee, JH.; Kim, DH. et al. (Mar 2013). "A case of colon perforation due to enteropathy-associated T-cell lymphoma.". World J Gastroenterol 19 (11): 1841-4. doi:10.3748/wjg.v19.i11.1841. PMID 23555174.
  6. ↑ Cairoli, A.; Ketterer, N.; Barelli, S.; Duchosal, MA. (Aug 2014). "High-dose therapy and autologous hematopoietic stem cell transplant in T-cell lymphoma: a single center experience.". Leuk Lymphoma 55 (8): 1827-31. doi:10.3109/10428194.2013.852666. PMID 24138331.
  7. ↑ Jantunen, E.; Boumendil, A.; Finel, H.; Luan, JJ.; Johnson, P.; Rambaldi, A.; Haynes, A.; Duchosal, MA. et al. (Mar 2013). "Autologous stem cell transplantation for enteropathy-associated T-cell lymphoma: a retrospective study by the EBMT.". Blood 121 (13): 2529-32. doi:10.1182/blood-2012-11-466839. PMID 23361910.
  8. ↑ 8.0 8.1 Jiao, G.; Zheng, Z.; Jiang, K.; Zhang, J.; Wang, B. (Jul 2014). "Enteropathy-associated T-cell lymphoma presenting with gastrointestinal tract symptoms: A report of two cases and review of diagnostic challenges and clinicopathological correlation.". Oncol Lett 8 (1): 91-94. doi:10.3892/ol.2014.2105. PMID 24959225.
  9. ↑ 9.0 9.1 9.2 Takeshita, M.; Nakamura, S.; Kikuma, K.; Nakayama, Y.; Nimura, S.; Yao, T.; Urabe, S.; Ogawara, S. et al. (Feb 2011). "Pathological and immunohistological findings and genetic aberrations of intestinal enteropathy-associated T cell lymphoma in Japan.". Histopathology 58 (3): 395-407. doi:10.1111/j.1365-2559.2011.03768.x. PMID 21323966.
  10. ↑ Kim, do H.; Lee, D.; Kim, JW.; Huh, J.; Park, SH.; Ha, HK.; Suh, C.; Yoon, SM. et al. (May 2014). "Endoscopic and clinical analysis of primary T-cell lymphoma of the gastrointestinal tract according to pathological subtype.". J Gastroenterol Hepatol 29 (5): 934-43. doi:10.1111/jgh.12471. PMID 24325295.
  11. ↑ Chan, JK.; Chan, AC.; Cheuk, W.; Wan, SK.; Lee, WK.; Lui, YH.; Chan, WK. (Oct 2011). "Type II enteropathy-associated T-cell lymphoma: a distinct aggressive lymphoma with frequent γδ T-cell receptor expression.". Am J Surg Pathol 35 (10): 1557-69. doi:10.1097/PAS.0b013e318222dfcd. PMID 21921780.
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