Overview of enteropathy type intestinal T-cell lymphoma
Enteropathy type T-cell lymphoma is a rare type of a very aggressive (fast growing) non-Hodgkin T-cell lymphoma (NHL). It is a subtype of PTCL. It grows in the small bowel. It develops from the white blood cells called T-cells (lymphocytes).
Types of enteropathy type intestinal T-cell lymphoma
There are two types of intestinal T-cell lymphoma. They are known by the characteristics that they show. They are called:
- Enteropathy-associated T-cell lymphoma (EATL): this subtype accounts for around 80% of all cases of intestinal T-cell lymphoma. ‘Enteropathy’ means ‘a disease of the intestines’. It used to be called ‘EATL type 1’.
- Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL): this subtype accounts for around 20% of all cases of intestinal T-cell lymphoma. It is called MEITL because the abnormal T-cells all look the same under the microscope (they are ‘monomorphic’). It grows in the lining of your intestine (your intestinal ‘epithelium’).
Who is affected?
Enteropathy type intestinal T-cell lymphomas normally affects older people, over the age of 60 years. Type one (now known as EATL) is strongly linked in people with untreated gluten-sensitive intestinal disease, called celiac disease. It is important to know that most people who have coeliac disease do NOT develop enteropathy type intestinal T-cell lymphoma.
Symptoms of enteropathy type intestinal T-cell lymphoma
The most common symptoms involve the stomach or bowel and may include:
- Abdominal pain
- Weight loss
- Diarrhoea, may have blood present
- An itchy rash
B symptoms may also include:
- Weight loss
- Drenching night sweats
Very occasionally a perforated (burst) bowel may happen. Malnourishment (not getting enough nutrients) could also be evident as the food may not be absorbed properly.
Diagnosis and staging of intestinal T-cell lymphoma
A biopsy is needed to diagnose intestinal type T-cell lymphoma and can be difficult to diagnose because the symptoms are similar to other conditions, including coeliac disease. The bowel is also difficult to see on scans.
An endoscopy is a diagnostic test where a thin tube is inserted into the mouth to examine the gastrointestinal tract and an endoscopy can be performed examine the bowel for anything that looks abnormal. Samples of tissue (a biopsy) from the gastrointestinal tract or the lining of the small bowel are collected for testing.
Intestinal T-cell lymphoma can be present in many different places in the bowel will not normally spread to other parts of the body. It is usually diagnosed at an early stage (stage 1 or 2). Many people who are diagnosed with intestinal T-cell lymphoma do become unwell with their symptoms and will need to be treated.
Prognosis of intestinal T-cell lymphoma
Intestinal T-cell lymphoma is difficult to treat as it is often diagnosed at a later stage and patients are often very unwell by the time it is diagnosed. It is also very rare, making it more difficult to do clinical trials for this patient group. Haematologists will work alongside the gastroenterologist to create a treatment plan that is best for the patient but is common for patients to relapse (comes back) after having the initial first-line treatment.
Treatment of intestinal T-cell lymphoma
This lymphoma is difficult to treat as patients are usually very unwell on diagnosis. Patients will need to be under the care of a haematologist and gastroenterologist as they will work together to plan the best possible treatment.
The most common standard first-line chemotherapy regimens may include:
- CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone)
- CHEOP (CHOP plus etoposide)
- Chemotherapy followed by autologous stem cell transplant
Common side effects of treatment
There are many different side effects of the treatment and these are dependent on the treatment that has been given. The treating doctor and/or cancer nurse can explain the specific side effects prior to the treatment. Some of the more common side effects of treatment may include:
- Anaemia (low red blood cells carry oxygen around the body)
- Thrombocytopenia (low platelets that help bleeding and clotting)
- Neutropenia (low white blood cells help with immunity)
- Nausea and vomiting
- Bowel problems such as constipation or diarrhoea
- Fatigue (tiredness or lack of energy
The medical team, doctor, cancer nurse or pharmacist, should provide information about:
- What treatment will be given
- What are the common and possible side effects for the treatment
- What side effects do you need to report to the medical team
- What are the contact numbers, and where to attend in case of emergency 7 days a week and 24 hours per day
Once treatment has completed, post treatment staging scans are done to review how well the treatment has worked. The scans will show the doctor if there has been a:
- Complete response (CR or no signs of lymphoma remain) or a
- Partial response (PR or there is still lymphoma present, but it has reduced in size)
If all goes well regular follow-up appointments will be made for every 3-6 months to monitor the below:
- Review the effectiveness of the treatment
- Monitor any ongoing side effects from the treatment
- Monitor for any late effects from treatment over time
- Monitor signs of the lymphoma relapsing
These appointments are also important so that the patient can raise any concerns that they may need to discuss with the medical team. A physical examination and blood tests are also standard tests for these appointments. Apart from immediately after treatment to review how the treatment has worked, scans are not usually done unless there is a reason for them. For some patient’s appointments may become less frequent over time.
Relapsed or refractory management intestinal T-cell lymphoma
For some patients, the initial treatment is very effective, and the lymphoma does not return after the treatment has finished. However, for some patients the lymphoma returns (relapses) or in rare cases does not respond to initial treatment (refractory). If this happens there are other treatments that can be successful including the following:
- High dose chemotherapy and autologous stem cell transplantation (own stem cells)
- High dose chemotherapy and allogeneic stem cell transplantation (donor stem cells)
- Combination chemotherapy
- Biological medicines such a romidepsin, brentuximab or pralatrexate
- Clinical trial participation
Treatment under investigation
There are many treatments that are currently being tested in clinical trials around the world and in Australia for patients with both newly diagnosed and relapsed lymphoma. Some of the current treatments that are being investigated for intestinal T-cell lymphoma include:
- Antibody-drug conjugates (brentuximab vedotin)
- Cell signal blockers (avelumab)
- Protesasome inhibitors (bortezomib or carfilzomib)
- HDAC inhibitors (vorinostat, romidepsin or belinostat)
What happens after treatment?
Sometimes a side effect from treatment may continue or develop months or years after treatment has completed. This is called a late effect.
This can be a challenging time for many people and some of the common concerns can be related to:
- Mental wellbeing
- Emotional health
- Work, study, and social activities
Health and wellbeing
A healthy lifestyle, or some positive lifestyle changes after treatment can be a great help after the treatment has been finished. Making small changes such as eating and increasing fitness can improve health and wellbeing and help the body to recover. There are many self-care strategies that can help during the recovery phase.