Overview of peripheral T-cell lymphoma – not otherwise specified (PTCL-NOS)
Peripheral T-cell lymphoma (PTCL) are a group of aggressive (fast growing) T-cell non-Hodgkin lymphomas (NHL). They account for 7% of all non-Hodgkin lymphoma (NHL) cases and 10% of all lymphoma cases.
Peripheral T-cell lymphoma – not otherwise specified (PTCL-NOS) is an aggressive (fast growing) T-cell non-Hodgkin lymphoma. This is the most common group of PTCL accounting for 35% of all T-cell lymphomas. PTCL-NOS refers to a group of diseases that do not fit into any of the other PTCL subtypes.
PTCL-NOS are aggressive and can be a nodal type that affects the lymph nodes. It can also be extranodal, that is located outside of the lymph nodes which may include the liver, bone marrow, intestinal tract and the skin. It can also be both nodal or extranodal and can be quite different from each other.
Who is affected by PTCL-NOS?
PTCL-NOS is the most common type of PTCL. It accounts for around a third of all cases. PTCL-NOS usually occurs in adults in the 50-60-year-old age group. This subtype affects more men than it does women.
Symptoms of peripheral T-cell lymphoma – NOS (PTCL-NOS).
Peripheral T-cell lymphoma – not otherwise specified (PTCL-NOS) is commonly found in the lymph nodes that can be anywhere in the body including, neck, armpit or groin. PTCL-NOS is most commonly found outside of the lymph nodes in other tissues or organs.
Common symptoms may include:
- Swollen lymph nodes-most common in the neck, armpit, or groin and are often found outside of the lymph nodes.
- Anaemia (low red blood cells) and thrombocytopenia (low platelets) as the bone marrow is often affected
- Bloating and abdominal pain due to enlarged spleen and liver
- Loss of appetite
- Itchy red patches on the skin
- Chest pain or shortness of breath if there is chest involvement
B symptoms are what doctors call certain symptoms and can include:
- Night sweats (drenching sleepwear and bedding)
- Persistent fever (especially at night >38C)
- Unexplained weight loss
- Itchy skin
It is important to tell the doctor about all symptoms as this can influence the decision around the type of treatment and when to start.
Prognosis of peripheral T-cell lymphoma – NOS (PTCL-NOS)
The current standard first-line treatment for peripheral T-cell lymphoma – not otherwise specified (PTCL-NOS) is chemotherapy. Patients commonly achieve remission (no signs of cancer detected) after first-line treatment. However, a high percentage of patients may relapse (the lymphoma returns) soon after but are ongoing research studies for PTCL-NOS to investigate new therapies to treat and improve outcomes.
Treatment for PTCL-NOS
Once all the results from the biopsy and the staging scans have been completed, the doctor will review these to decide the best possible treatment for a patient. At some cancer centres, the doctor will also meet with a team of specialists to discuss the best treatment and this is called a multidisciplinary team (MDT)meeting.
Doctors take into consideration many factors about the lymphoma and the patient’s general health to decide when and what treatment is required.
This is based on:
- The stage of lymphoma
- Symptoms (including the size and location of the lymphoma)
- How the lymphoma is affecting the body
- Past medical history & general health
- Current physical and mental wellbeing
- Patient preferences
The initial standard first-line treatment protocol for PTCL-NOS can include:
- CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone)
- CHOEP (CHOP with addition of etoposide)
- Chemotherapy followed by autologous stem cell transplant (ASCT)
- Clinical trial participation
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
Some treatments for lymphoma can reduce fertility and thisis more likely to occur with certain treatments. These may include some chemotherapy protocols (combinations of drugs) and the high-dose chemotherapy used before a stem cell transplant. Radiotherapy to the pelvis also increases the likelihood of reduced fertility and some antibody therapies may also affect fertility, but this is less clear.
The doctor should advice before treatment begins if fertility may be affected and whether fertility preservation should be done. This should be done before you start treatment.
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 PTCL-NOS
Peripheral T-cell lymphoma (PTCL) usually responds well to immunochemotherapy. In some people the lymphoma comes back (relapses) and in rare cases the lymphoma does not respond to initial treatment and is termed (refractory). However, there are other treatments that can be given, and these include:
- High dose chemotherapy and autologous stem cell transplantation (ASCT)
- High dose chemotherapy and allogeneic stem cell transplantation (ASCT)
- Combination chemotherapy
- Biological medicines, such as romidepsin, Brentuximab or pralatrexate
- Clinical trial participation
Treatment under investigation
PTCL-NOS is known to relapse after being in remission and various targeted therapies are being tested for PTCL-NOS. A number of clinical trials may be available and the therapies can include:
- Brentuximab vedotin
What happens after treatment?
Sometimes a side effect from treatment may continue or develop months or years after treatment has completed.
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.