Non-Hodgkin lymphoma

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Enteropathy-associated T-cell lymphoma

Enteropathy-associated T-cell lymphoma (EATL) is a very rare type of non-Hodgkin lymphoma (NHL). It may also be called enteropathy-type T-cell lymphoma or intestinal T-cell lymphoma. It is a fast-growing (aggressive) T-cell lymphoma.

EATL is more common in Europe and in men. It occurs most often in people 60 to 70 years old.

EATL develops most often in the middle part of the small intestine (called the jejunum). It can also develop in other parts of the small intestine and in the colon (the longest part of the large intestine). EATL often affects more than one place in the intestine. It may spread to the liver, spleen, lymph nodes, lungs, gallbladder or stomach.

Types of enteropathy-associated T-cell lymphoma

Type I EATL is linked to celiac disease or gluten sensitivity. Gluten is a protein found in many grains, such as wheat, rye and barley. When people with celiac disease or gluten sensitivity eat food with gluten, their body reacts to it. This reaction results in damage to the lining of the small intestine. When people are diagnosed with celiac disease or gluten sensitivity, they follow a gluten-free diet. This helps prevent EATL. As a result, type I EATL does not commonly occur in people diagnosed with celiac disease at a young age. Most adults are diagnosed with celiac disease at the same time as, or shortly before, their EATL is diagnosed.

Type II EATL is not linked to celiac disease or gluten sensitivity. It is less common than type I EATL.

Symptoms

People with EATL often have:

  • ulcers in the small intestine
  • a blockage in the small intestine
  • a hole in the wall of the small intestine (called bowel perforation)

All of these problems can cause severe abdominal pain, nausea and vomiting, weight loss and blood in the stool.

Treatments

There is no standard approach to treating EATL. The treatments offered are based on where it started in the small intestine or colon and where it has spread.

Surgery

Surgery is often the first treatment for EATL. The surgeon removes the part of the small intestine or colon that contains the cancer. Samples of the tissue removed are sent to the lab to confirm that it is EATL. Surgery to remove the cancer also helps prevent a bowel perforation during treatment.

Chemotherapy

Chemotherapy may be offered after surgery. It is also used as the main treatment instead of surgery if EATL is advanced or it can’t be completely removed with surgery.

A combination of chemotherapy drugs that includes doxorubicin (Adriamycin) may be given. The most common combination used is CHOP:

  • cyclophosphamide (Cytoxan, Procytox)
  • doxorubicin
  • vincristine (Oncovin)
  • prednisone

Sometimes high-dose methotrexate is used alone for EATL.

Stem cell transplant

A stem cell transplant may be an option for some people during remission or when EATL comes back (recurs) after treatment.

remission

A decrease in or the disappearance of signs and symptoms of a disease (such as cancer).

Complete remission means the disappearance of all signs or symptoms. Partial remission means a decrease in or disappearance of some, but not all, signs and symptoms. Spontaneous remission is an unexpected improvement that occurs with little or no treatment.

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