Enteropathy-associated T-cell lymphoma

Enteropathy-associated T-cell lymphoma
ICD-O-2 Morphology
Effective 1995 - 2000
ICD-O-3 Morphology
9717/3: Enteropathy-associated T-cell lymphoma
Effective 2001 and later
for cases diagnosed 1995 and later
Primary Site(s)
See Module 7
Most common sites of involvement: jejunum, ileum, duodenum, stomach, colon, outside of GI tract (rare)

Not Applicable
Module Rule
Alternate Names
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)
A tumor of intraepithelial T-lymphocytes, showing varying degrees of transformation but usually presenting as a tumor composed of large lymphoid cells. Tumor most commonly occurs in the jejunum or ileum. This disease is most commonly found in the setting of celiac disease.

Often preceded by celiac disease (gluten sensitive enteropathy) which is sometimes accompanied by intestinal ulceration (ulcerative jejunitis), but this is not a true "precursor" or "transformation."

In 10-20% of cases the lymphoma is composed of monomorphic medium-sized cells. This monorophic variant may be called Type II EATL.
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).

The monomorphic variant appears to have a broader geographic distribution and is encountered in Asia and other regions where celiac disease is rare. The tumor forms an ulcerating mucosal mass that invades the wall of the intestine. The prognosis is usually poor with death frequently resulting from abdominal complications in patients already weakened by uncontrolled malabsorption.

Both classical EATL and the monomorphic form have a similar clinical course.
Definitive Diagnostic Methods
Genetic testing
Histologic confirmation
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
Positive/Negative: CD8, TCR beta
Stem cell transplant
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