Lymphoma & related disorders

Mature T/NK cell disorders

Intestinal

Enteropathy associated



Topic Completed: 20 August 2021

Minor changes: 20 August 2021

Copyright: 2001-2021, PathologyOutlines.com, Inc.

PubMed search: Enteropathy associated T cell lymphoma [title] intestinal

Mario L. Marques-Piubelli, M.D.
Roberto N. Miranda, M.D.
Page views in 2020: 4,073
Page views in 2021 to date: 3,401
Cite this page: Marques-Piubelli ML, Miranda RN. Enteropathy associated. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomanonBenteropathy.html. Accessed October 20th, 2021.
Definition / general
  • Intestinal lymphoma derived from intraepithelial T cells which occurs in patients with celiac disease (Blood 2011;118:148)
Essential features
Terminology
  • Enteropathy associated T cell lymphoma type I (obsolete)
  • Intestinal T cell lymphoma
  • Enteropathy type T cell lymphoma (obsolete)
ICD coding
  • ICD-O: 9717/3 - intestinal T cell lymphoma
  • ICD-10: C86.2 - enteropathy type (intestinal) T cell lymphoma
Epidemiology
Sites
Etiology
Pathophysiology
Clinical features
Diagnosis
Laboratory
Radiology description
  • 18F-Fluoro-deoxy-glucose positron emission tomography (18F-FDG-PET) (Gut 2003;52:347)
    • Patients with long standing celiac disease and a standard uptake value > 6 need further investigation for EATL
  • Computed tomography (CT) has a higher accuracy than small bowel enema (Blood 2012;119:2458)
    • Findings that increase the suspicion for EATL
      • Bowel wall thickening
      • Mesenteric lymph node cavitation
      • Intussusception
      • Small sized spleen (< 120 cm3)
    • Video capsule endoscopy (Curr Hematol Malig Rep 2016;11:504)
      • May determine the extent and the precise location of the lesions
      • Useful in cases of intestinal bleeding
Staging
  • Staging procedures as performed in other lymphomas (Blood 2012;119:2458)
    • Bone marrow examination
    • CT of thorax, head and neck
  • Patients usually present with advanced stage of disease (Blood 2011;118:148)
Prognostic factors
  • Poor prognosis (Blood 2011;118:148)
    • Median overall survival (OS): 10 months
    • 5 year overall survival: 20%
  • Prognostic index for peripheral T cell lymphoma (PIT score)
    • Predictive of survival
    • Parameters: lactate dehydrogenase (LDH), age, bone marrow involvement and performance status
  • Factors associated with better overall survival (Dig Liver Dis 2013;45:377):
    • Serum albumin level > 21.6 g/L
    • Chemotherapy treatment
    • Surgical resection
  • Factors associated with worse overall survival (Blood 2011;118:148):
    • Large tumor mass (> 5 cm)
    • Nonambulatory performance status
    • Elevated serum lactate dehydrogenase and C reactive protein (CRP)
Case reports
Treatment
  • Surgery (Blood 2012;119:2458)
    • Debulking and resection of tumor mass
    • Role in complications, mainly in cases of obstruction and perforation
  • Standard dose combination chemotherapy (Blood 2011;118:148, Blood 2012;119:2458)
    • Less than 50% achieve complete remission
    • Common regimes
      • Cyclophosphamide, doxorubicin, vincristine, prednisolone (CHOP) or CHOP-like regimen
        • Patients who do not respond to first line CHOP usually have poor prognosis
      • Bleomycin, doxorubicin, cyclophosphamide, vincristine and prednisone (BACOP)
      • Vincristine, doxorubicin, high dose methotrexate and prednisolone (VAMP)
      • Prednisolone, doxorubicin, cyclophosphamide, etoposide, mechlorethamine, vincristine and procarzabine (ProMACE-MOPP)
      • Cisplatin, cytarabine, etoposide and methylprednisolone (ESHAP)
      • Carmustine, etoposide, cytarabine and melphalan (BEAM)
  • Combination of high dose chemotherapy followed by consolidation with autologous stem cell transplantation consolidation (Blood 2011;118:148, Blood 2013;121:2529)
    • If patient is eligible, may lead to long term remission
  • Radiation therapy is controversial (Blood 2012;119:2458)
  • Novel treatments (Blood 2012;119:2458)
    • Alemtuzumab
    • Cladribine
    • Romidepsin
Gross description
Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Roberto N. Miranda, M.D.
Ileocecal ulcer

Ileocecal ulcer

Ileocecal infiltration

Ileocecal infiltration

Angiocentric and angioinvasive lesion

Angiocentric and angioinvasive lesion

Mucosal and  submucosal infiltration

Mucosal and submucosal infiltration


Cytologic atypia

Cytologic atypia

Intraepithelial lymphocytes

Intraepithelial lymphocytes

Sinusoidal infiltration

Sinusoidal infiltration

Sinusoidal infiltration

Sinusoidal CD30 positivity


CD3 positivity

CD3 positivity

CD30 positivity

CD30 positivity

CD4 negativity

CD4 negativity

CD8 negativity

CD8 negativity

TIA1 positivity

TIA1 positivity

Positive stains
Molecular / cytogenetics description
Sample pathology report
  • Small intestine, jejunum biopsies:
    • Enteropathy associated T cell lymphoma (see comment)
    • Comment:
      • According to clinical notes, the patient has a previous history of celiac disease diagnosed 7 years ago and recently started with anemia, weight loss and diarrhea. Endoscopic examination showed multiple and active ulcers raising mucosal masses in jejunum and mosaic pattern on the adjacent mucosae. A computed tomography realized on the same day showed enlarged mesenteric lymph nodes.
      • Multiple biopsies from the jejunum are reviewed. Hematoxylin and eosin sections show an ulcerated lesion and dense infiltration in mucosae and submucosae by atypical lymphocytes. The abnormal lymphocytes are of medium to large size, with prominent nucleoli and moderate to abundant cytoplasm and surrounded by a background with occasional histiocytes, eosinophils and neutrophils. The mucosa adjacent to the atypical infiltrate showed numerous small intraepithelial lymphocytes. Immunohistochemical studies demonstrated that the neoplastic cells are positive for CD3, CD7, TIA1 and TCR-β. The neoplastic cells are negative for CD4, CD5, CD8, CD20, CD56, PAX5, TCRγδ and EBER. The adjacent intraepithelial lymphocytes show a similar reactivity to the lymphoma cells.
      • Concurrent polymerase chain reaction for T cell receptor gamma chain demonstrated monoclonal gene rearrangement.
      • In summary, the clinicopathologic findings support a diagnosis of enteropathy associated T cell lymphoma in a patient with celiac disease.
Differential diagnosis
Board review style question #1
Which of the following is true about enteropathy associated T cell lymphoma (EATL)?

  1. It does not associate with celiac disease
  2. Large and unifocal mass is the most common endoscopic finding
  3. There is an association with HLA-DQ2.2, HLA-DQ5 and HLA-DQ8
  4. Usually presents with peripheral lymphadenopathy
Board review style answer #1
C. There is an association with HLA-DQ2.2, HLA-DQ5 and HLA-DQ8. EATL is characteristically associated with celiac disease and in particular persons with major histocompatibility complex expressing HLA-DQ2.2, HLA-DQ5 and HLA-DQ8. Generalized lymphadenopathy is not a feature of intestinal lymphomas. Endoscopically, multiple small ulcerated lesions are noted.

Comment Here

Reference: Enteropathy associated T cell lymphoma (EATL)
Board review style question #2

What is the most consistent immunophenotype of enteropathy associated T cell lymphoma (EATL)?

  1. CD3+, CD4+, CD5+, CD7+, CD8-, EBER+, TCR-β-
  2. CD3+, CD4-, CD5-, CD7+, CD8-, EBER-, TCR-β-
  3. CD3+, CD4-, CD5+, CD7-, CD8-, EBER+, TCR-β+
  4. CD3+, CD4+, CD5-, CD7+, CD8-, EBER-, TCR-β-
Board review style answer #2
B. CD3+, CD4-, CD5-, CD7+, CD8-, EBER-, TCR-β-. EATL is a T cell lymphoma and thus expresses CD3, usually lacks CD4 and CD8 as well as TCR-β and is negative for Epstein-Barr virus.

Comment Here

Reference: Enteropathy associated T cell lymphoma (EATL)
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