Enteropathy-associated T-cell lymphoma

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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
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

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 due to celiac sprue.
  • Subdivided into type I and type II.[1]

Gross

Features:[2]

  • Typically manifest as ulcers, fissures or plaques. †

Notes:

  • † B-cell lymphomas are typically raised nodules.[2]

Microscopic

Features:[3]

  • 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, e.g. DLBCL.

Image

  • EATL. (WC)

IHC

Features - type 1:[3]

Features - type 2:[3][4]

  • CD3 +ve.
  • CD5 -ve.
  • CD8 -ve/+ve.
  • CD56 -ve/+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 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.
  3. ↑ 3.0 3.1 3.2 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.
  4. ↑ 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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