T-cell lymphomas are very rare. PTCL-NOS is a type of T-cell lymphoma – a non-Hodgkin lymphoma that develops from white blood cells called T cells. PTCL-NOS includes all T-cell lymphomas that don’t fit into any other category. It is one of the more common types of these rare T-cell lymphoma.
The lymphomas within this group can be quite different from each other. However, they might share some common symptoms. As more research is done in the future, it is likely that PTCL-NOS will be split into several subtypes of T-cell lymphoma rather than being discussed as a single type of lymphoma.
T-cell lymphoma is a very rare type of lymphoma. PTCL-NOS is the most common T-cell lymphoma, accounting for around 1 in 3 cases. It can occur at any age, but it is more common in people in their 60s. It tends to affect more men than women. Most people have advanced stage lymphoma when they are diagnosed.
The most common symptom people with PTCL-NOS might have is a swollen lymph node or nodes. These can develop anywhere in the body but the most common places are the neck, armpit or groin. PTCL-NOS is also commonly found outside of the lymph nodes (extranodal areas).
- The bone marrow (the spongy tissue in the centre of bones where blood cells are made) is often affected, which can lead to anaemia (low red blood cells) and thrombocytopenia (low platelet levels).
- The liver and spleen might be enlarged, causing bloating or tummy (abdominal) pain. Lymphoma elsewhere in your gut might cause pain, sickness, diarrhoea or vomiting.
- The skin can be affected by itchy red patches.
- If you have lymphoma in your chest, you might feel short of breath, develop a cough or have pain or a feeling of pressure in your chest.
Fevers, night sweats and unexplained weight loss (known as ‘B symptoms’) are also common in people with PTCL-NOS.
PTCL-NOS is the most common type of T-cell lymphoma, but like all T-cell lymphomas, it is still rare. This makes it difficult for doctors to determine which treatment will give you the best outcome.
There are no standard treatments for PTCL-NOS. Several targeted drugs are being tested for PTCL-NOS and different chemotherapy regimens (combinations of drugs) are being compared in clinical trials. Your doctor might ask you if you would like to take part in a clinical trial to test new treatments and help find out what the best treatment is for PTCL-NOS.
If you don’t want to take part in a clinical trial, or if there isn’t one that is suitable for you, you are likely to be treated with chemotherapy. The most common chemotherapy regimen used for PTCL-NOS is CHOP:
- doxorubicin (or hydroxydaunorubicin)
- vincristine (also known as Oncovin®)
- prednisolone (a steroid).
Your lymphoma specialist might also recommend a chemotherapy drug called etoposide in addition to CHOP (CHEOP), or you might have a different chemotherapy regimen altogether. If you are not fit enough to have CHOP or CHEOP, you might be treated with less intensive chemotherapy, such as gemcitabine or bendamustine.
If you respond to chemotherapy and you are well enough, your doctor is likely to recommend that you have a self (autologous) stem cell transplant. This gives you a better chance of staying in remission (no evidence of lymphoma) after treatment.
PTCL-NOS might respond well to initial treatment, but it is common for it to come back (relapse) after either an initial response or having achieved a complete remission. However sometimes, PTCL-NOS fails to respond to treatment (refractory lymphoma). In these cases, your doctor might consider:
- a different chemotherapy regimen, such as GDP (Gemcitabine, Dexamethason, Platinum), GemOx (Gemcitabine, Oxaliplatin), DHAP (dexamethasone, high-dose cytarabine [also known as Ara-C] and cisplatin [also known as Platinol®]) or ICE (ifosfamide, carboplatin and etoposide)
- a donor (allogeneic) stem cell transplant if your lymphoma responds to more chemotherapy and you are well enough
- a targeted drug, usually through a clinical trial.
Many targeted drugs are being tested to see if they can help people with T-cell lymphoma. Drugs that have been or are being tested in PTCL-NOS include:
- antibody–drug conjugates such as brentuximab vedotin
- immune checkpoint inhibitors such as avelumab and pembrolizumab
- proteasome inhibitors such as bortezomib or carfilzomib
- HDAC inhibitors such as vorinostat, romidepsin or belinostat
- a chemotherapy drug called pralatrexate
- antibody treatments such as alemtuzumab, which binds to a protein called CD52 on T cells.
Some of these might be available to you through a clinical trial. If you are interested in taking part in a clinical trial, ask your doctor if there is a trial that might be suitable for you. To find out more about clinical trials or search for a trial that might be suitable for you, visit Lymphoma TrialsLink.