Enteropathy-associated T-cell lymphoma C86.2

Last updated on: 09.10.2022

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Definition
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Aggressive non-Hodgkin's lymphoma of the gastrointestinal tract, often associated with a gluten-sensitive enteropathy (celiac disease). The tumor arises from intestinal intraepithelial cytotoxic T cells. The small intestine and the mesentery are most frequently affected; other localizations in the gastrointestinal tract are less frequent.

Classification
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In older classifications, according to:

  • EATL type 1: associated with gluten sensitive enteropathy in 80-90%, CD56 negative (EATZL often develops from EATL type 2, but can also occur "de novo" in individuals with celiac disease (Chander U et al. 2018).
  • EATL type 2: in 10-20% no association with gluten-sensitive enteropathy, CD56-positive (new nomenclature: monomorphic epitheliotropic intestinal T-cell lymphoma).

Classified.

According to the current WHO classification of 2017, the name "EATL" is used only for the previous type 1.

Occurrence/Epidemiology
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Rare disease; <5% of all gastrointestinal lymphomas and <1% of all non-Hodgkin lymphomas are enteropathy-associated T-cell lymphomas

Manifestation
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Occurrence mostly in the 6th to 7th decade of life

Clinical features
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Abdominal pain (most common symptom); furthermore, symptoms of gluten-sensitive enteropathy are detectable such as steatorrhea, flatulence, weight loss, malabsorption, gastrointestinal hemorrhage, anemia, B symptoms, intestinal obstruction, or intestinal perforation (Chander U et al. 2018).

A large proportion of patients present with advanced stage disease. Many patients are not diagnosed with gluten-sensitive enteropathy until enteropathy-associated T-cell lymphoma is also diagnosed.

Cutaneous manifestations are possible in the setting of systemic lymphoma. Although the clinical picture raises a suspicion of cutaneous lymphoma, it can otherwise only be verified by the histological and molecular biological pattern (Bisig B et al. 2022) .

Histology
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Evidence of T-cell clonality, tumor cells of variable size and, depending on the type, expression of various receptors such as CD56 or CD8. Genetically, ETL often show chromosomal gains from 9q33-q34. The detection of recurrent activating mutations in members of the JAK/STAT pathway, may suggest that deregulation of cytokine signaling is an early event in lymphomagenesis.

Therapy
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Consistent gluten-free diet, steroids in patients with EATL type 1 and refractory symptoms of gluten-sensitive enteropathy.

Chemotherapy not yet standardized, participation in trials recommended; usually CHOP regimen, if necessary with an additional application administration of etoposide (in patients < 60 years).

Autologous stem cell transplantation is also a therapeutic option.

Progression/forecast
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The tumor behaves aggressively. Frequent metastasis to the liver, spleen, skin and other organs. Typical complication is intestinal perforation. The median survival rate from diagnosis is 10 months.

Prophylaxis
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Consistent gluten-free diet can prevent the development of the disease.

Note(s)
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Gastrointestinal lymphomas are almost exclusively non-Hodgkin lymphomas (NHL). They arise somewhat more frequently in the stomach than in the intestine and mostly originate from B cells. Aggressive NHL, especially diffuse large B-cell lymphoma or enteropathy-associated T-cell lymphoma, are more frequently found in the gastrointestinal tract than indolent lymphomas (De Leval L et al. 2015).

Literature
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  1. Bisig B et al (2022) Cutaneous presentation of enteropathy-associated T-cell lymphoma masquerading as a DUSP22-rearranged CD30+ lymphoproliferation. Virchows Arch 481:653-657
  2. Chander U et al (2018) Pathogenesis of enteropathy-associated T cell lymphoma. Curr Hematol Malig Rep13:308-317.
  3. De Leval L et al (2015) Recent advances in intestinal lymphomas. Histopathology 66: 112-135.
  4. Ganapathi KA et al. (2014) Early lymphoid lesions: conceptual, diagnostic and clinical challenges. Haematologica 99: 1421-1432.
  5. Luchtel RA et al (2018) Molecular profiling reveals immunogenic cues in anaplastic large cell lymphomas with DUSP22 rearrangements. Blood 132:1386-1398
  6. Malamut G et al.(2014) Small intestinal CD4+ T-cell lymphoma is a heterogeneous entity with common pathology features. Clin Gastroenterol Hepatol 12: 599-608.
  7. Margolskee E et al (2013) Indolent small intestinal CD4+ T-cell lymphoma is a distinct entity with unique biologic and clinic features. PLoS One 8: e68343.
  8. Perry AM et al (2013) Indolent T-cell lymphoproliferative disease of the gastrointestinal tract. Blood 122: 3599-3606
  9. Takeuchi K et al.(2010) Lymphomatoid gastropathy: A distinct clinicopathologic entity of self-limited pseudomalignant NK-cell proliferation. Blood 116: 5631-5637.

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Last updated on: 09.10.2022