Enteropathy-associated T-cell lymphoma

From Libre Pathology
Jump to navigation Jump to search
The printable version is no longer supported and may have rendering errors. Please update your browser bookmarks and please use the default browser print function instead.
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

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.