Name

Enteropathy-associated T-cell lymphoma

ICD-O-3 Morphology

9717/3: Enteropathy-associated T-cell lymphoma
Effective 2001 and later

Reportable

for cases diagnosed 2001 and later

Primary Site(s)

See Module 7
Most common sites of involvement: jejunum, ileum, duodenum, stomach, colon, outside of GI tract (rare)

Grade

Not Applicable

Module Rule

None

Alternate Names

Classic enteropathy-associated T-cell lymphoma
EATL
Enteropathy type intestinal T-cell lymphoma
Enteropathy-type peripheral T-cell lymphoma
Enteropathy-type T-cell lymphoma
Intestinal T-cell lymphoma (with and without enteropathy)
Malignant histiocytosis of the intestine [OBS]
Monomorphic epitheliotropic intestinal T-cell lymphoma
Type II enteropathy associated T-cell lymphoma

Definition

Enteropathy-associated T-cell lymphoma (EATL), previously designated type I EATL, is a neoplasm of intraepithelial T cells that occurs in individuals with celiac disease and exhibits varying degrees of cellular pleomorphism. It commonly presents as a tumor composed of medium-sized to large lymphocytes often accompanied by a component of chronic inflammatory cells. The adjacent small intestinal mucosa shows villous atrophy, crypt hyperplasia, and increased intraepithelial lymphocytes.

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a primary intestinal T-cell lymphoma derived from intraepithelial lymphocytes. Unlike in the classic form of enteropathy-associated T-cell lymphoma (EATL), there is no clear association with celiac disease. The neoplastic cells have medium-sized round nuclei with a rim of pale cytoplasm. There is usually florid infiltration of intestinal epithelium. An inflammatory background is absent, and necrosis is usually less evident than in classic EATL.

Intestinal T-cell lymphoma, NOS is used for T-cell lymphomas arising in the intestines, or sometimes other sites in the gastrointestinal tract, that do not conform to either classic enteropathy-associated T cell lymphoma or monomorphic epitheliotropic intestinal T-cell lymphoma. Sometimes this diagnosis is made based on an inadequate biopsy in which the mucosal surface cannot be evaluated or immunophenotype data are incomplete. It is not considered a specific disease entity.

Abstractor Notes

A small proportion of the patients have a history of childhood onset celiac disease. Most show adult onset disease or are diagnosed as having celiac disease in the same clinical episode in which the lymphoma is diagnosed. In a proportion of patients there is a prodromal period of refractory celiac disease that is sometimes accompanied by intestinal ulceration (ulcerative jejunitis).

Both classical EATL and the monomorphic form have a similar clinical course.

Definitive Diagnostic Methods

Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

8q24(MYC) amplifications (monomorphic variant)
Chromosome gains in 1q and 5q
Complex segmental amplifications of 9q31.3-qter chromosome region
Deletions 16q12.1
HLADQA1*0501, DQB1*0201 genotype
T-cell receptor (TCR) Beta and Gamma (TRB@, TRG@) genes are clonally rearranged

Immunophenotyping

CD3 positive
CD4 negative
CD5 negative
CD7 positive
CD8 negative
CD30 expression
CD103 positive
Granzyme B expressed
Perforin expressed
TIA1 expressed

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy
Radiation therapy
Surgery

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Corresponding ICD-9 Codes

202.8 Other lymphoma

Corresponding ICD-10 Codes

C84.5 Other and unspecified T-cell lymphomas

Corresponding ICD-10-CM Codes (U.S. only)

C86.2 Enteropathy-type (intestinal) T-cell lymphoma (effective October 01, 2015)

Signs and Symptoms

Abdominal pain (primarily due to intestinal perforation)
Drenching night sweats
Fever (for no known reason)
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Skin rash or itchy skin
Ulcerative jejunitis
Weight loss (for no known reason)

Diagnostic Exams

Progression and Transformation

Recurrences most frequent in the small intenstine

Epidemiology and Mortality

Country: Northern Europe, especially for patients with celiac disease
Survival: poor, death usually results from abdominal complications

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature T- and NK-cell neoplasms
Pages: 372-378

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary