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Enteropathy-Associated T cell Lymphoma.

Zakiah Al Somali1, Mehdi Hamadani2, Mohamed Kharfan-Dabaja3

  • 1Adult Hematology/HSCT, Oncology Center, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia.

Current Hematologic Malignancy Reports
|May 19, 2021
PubMed
Summary

Enteropathy-associated T cell lymphoma (EATL) is a rare, aggressive cancer. Early diagnosis, adherence to gluten-free diets for celiac disease patients, and clinical trials are crucial for improved outcomes.

Keywords:
Celiac diseaseEATLIntestinal lymphomaT cell Lymphoma

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Area of Science:

  • Hematology
  • Oncology
  • Gastroenterology

Background:

  • Enteropathy-associated T cell lymphoma (EATL) is a rare mature T cell lymphoma with limited literature.
  • It is primarily associated with celiac disease (CD) and Northern European populations.
  • EATL accounts for less than 5% of peripheral T cell lymphomas (PTCL).

Purpose of the Study:

  • To review and summarize current literature on EATL.
  • To discuss risk factors, pathogenesis, diagnosis, and treatment options for EATL.
  • To highlight the aggressive nature and poor prognosis of EATL.

Main Methods:

  • Literature review and synthesis of available research on EATL.
  • Analysis of risk factors including age, sex, and HLA-DQ2 homozygosity.
  • Evaluation of diagnostic standards and therapeutic strategies.

Main Results:

  • EATL has two subtypes, with Type I EATL strongly linked to celiac disease.
  • Pathogenesis shares features with refractory celiac disease.
  • Histological diagnosis is the gold standard; EATL has an aggressive course and poor prognosis.

Conclusions:

  • Current treatments yield poor outcomes; clinical trials are recommended.
  • Early diagnosis, referral to specialized centers, and new prognostic models are needed.
  • Prevention through gluten-free diet adherence in CD patients and surveillance is paramount.