Peripheral T-cell lymphoma (PTCL)

Peripheral T-cell lymphomas (PTCL) is the name given to the group of aggressive (fast growing) T-cell non-Hodgkin lymphomas.

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Peripheral T-cell lymphoma Fact Sheet

Overview of peripheral T-cell lymphoma (PTCL)

Peripheral T-cell lymphomas (PTCL) is the name given to the group of aggressive (fast growing) T-cell non-Hodgkin lymphomas. PTCL develops from a certain type of cell called T-lymphocytes. They account for 7% of non-Hodgkin lymphoma (NHL) cases and 10% of all lymphoma cases.

PTCL develops from mature-stage T-cells and natural killer (NK) cells. ’Peripheral’ does not refer to the extremities. It refers to a cancer that arises in the lymphoid tissue, but outside of the bone marrow. This includes lymph nodes, spleen, gastrointestinal tract, and skin.

There are more than 29 different subtypes of PTCL. Each subtype of PTCL is very rare and the most aggressive (fast-growing) in behaviour. There are exceptions that include some forms of cutaneous T-cell lymphoma (CTCL). These can be indolent (slow-growing) in behaviour. There are 4 main types of PTCL that include cutaneous, extranodal, nodal and leukaemic.

Who is affected by peripheral T-cell lymphoma (PTCL)?

Peripheral T-cell lymphoma (PTCL) affects both men and women.  It can also affect children and young people.  PTCL most commonly presents in patients over 60 years of age.  The cause of PTCL is mostly unknown and you are unable to catch it from other people.

Symptoms of peripheral T-cell lymphoma (PTCL)

The most common first sign of peripheral T-cell lymphoma (PTCL) is a painless lump.  The lump can grow in the neck, armpit, or groin.  The swelling is caused by the lymph nodes due to the presence of the lymphoma. For some patients, this swelling may be uncomfortable.  This is because the lymph node might be pressing on an area of the body causing pain. Symptoms generally depend on where in the body the lymphoma is located. Other symptoms may include:

  • Painless swelling or lump, mainly in the neck, groin or armpit
  • Loss of appetite
  • Shortness of breath
  • Stomach or abdominal discomfort or pain
  • Pain
  • Fatigue

B symptoms are symptoms that some patients may have 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.

Diagnosis and staging of peripheral T-cell lymphoma (PTCL)

biopsy is always required for diagnosis of lymphoma. A biopsy is a surgical procedure to remove part of or all an affected lymph node or other tissue.   The pathologist will then look under the microscope to see what the cells look like.  The biopsy can be done under local or a general anaesthetic.  This will depend on what part of the body is being biopsied. The biopsy can be taken one of three ways:

  • Fine needle aspirate
  • Core needle biopsy
  • Excisional node biopsy

An excisional node biopsy is the best investigative option, as it collects the most adequate amount of tissue to be able to do the necessary testing for a diagnosis.

Waiting for results can be a difficult time.  It may help to talk to family, friends or a specialist nurse. 

For more info see
Diagnosis of Lymphoma

Staging of peripheral T-cell lymphoma (PTCL)

Once a diagnosis of peripheral T-cell lymphoma – not otherwise specified (PTCL-NOS) is made, further tests will need to be done.  This is to see where else in the body the lymphoma has affected or is located.  This is called staging.  The staging of lymphoma helps the doctor determine the best treatment for the patient.

There are four stages from stage 1 (lymphoma in one area) through to stage 4 (lymphoma that is widespread). 

  • Early stage means stage 1 and some stage 2 lymphoma.  Also referred to as ‘localised’.  Stage 1 or 2 means that the lymphoma is found in one area or a few areas close together.
  • Advanced stage means that the lymphoma is stage 3 or stage 4, and it is widespread lymphoma.  In most cases, the lymphoma has spread to parts of the body that are far from each other.

At diagnosis, most people with peripheral T-cell lymphoma (PTCL), have advanced stage disease.  This is because PTCL is a fast-growing lymphoma.

What is the ‘grade’ of lymphoma?

Lymphomas are also often grouped as either indolent (slow growing) or aggressive (fast growing. The grade is also referred to as the clinical behaviour of the lymphoma.  PTCL is usually a high-grade lymphoma.  

It is important to know the stage and grade of the lymphoma as this will help the doctor to determine the best treatment for the patient.

For more info see
Staging and Grading

Staging scan and tests

The scans and tests needed for staging and before treatment can start may include:

  • Positron emission tomography (PET) scan 
  • Computed tomography (CT) scan 
  • Bone marrow biopsy 
  • Lumbar puncture & magnetic resonance imaging (MRI) – If lymphoma is suspected in the brain or spinal cord

Patients may also undergo a number of baseline tests prior to any treatment commencing to check organ functions.  These are often repeated during and after the treatment has completed to assess whether the treatment has affected the functioning of organs.  Sometimes the treatment and follow-up care may need to be adjusted to help manage side effects. These may include:

  • Physical examination
  • Vital observations (blood pressure, temperature, & pulse rate)
  • Heart scan
  • Kidney scan
  • Breathing tests
  • Blood tests

It may take some time for all the necessary biopsies and tests to be done (an average of 1-3 weeks), but it is important for the doctors to have a complete picture of the  lymphoma and the general  health of the patient  in order to make the best treatment decisions 

Many of the staging and organ function tests are done again after treatment to check whether the lymphoma treatment has worked and the effect this has had on the body.

For more info see
Staging Scans & Tests

Types of peripheral T-cell lymphoma (PTCL)

There are more than 29 different subtypes of peripheral T-cell lymphoma (PTCL). Each subtype of PTCL is very rare and mostly aggressive (fast-growing) in behaviour.  Yet, some forms of cutaneous T-cell lymphoma (CTCL) can be indolent (slow-growing).  There are 4 main types of PTCL that include cutaneous, extranodal, nodal and leukaemic.

Nodal T-cell lymphoma

Nodal T-cell lymphomas are found in the lymph nodes.  Different subtypes include:

  • Peripheral T-cell lymphoma – not otherwise specified (PTCL-NOS)
  • Angioimmunoblastic T-cell lymphoma (AITL)
  • Anaplastic large cell lymphoma (ALCL)

Extranodal T-cell lymphoma

Extranodal T-cell lymphomas are found outside of the lymph nodes.  Different subtypes include:

  • Intestinal T-cell lymphoma
    • Enteropathy-Type T-Cell Lymphoma (EATL)
    • Monomorphic epitheliotropic intestinal lymphoma (MEITL)
  • Nasal NK/T-Cell Lymphoma (NKTCL)
  • Hepatosplenic gamma delta T-cell lymphoma

Cutaneous T-cell lymphoma

Cutaneous T-cell lymphomas are found in the skin tissue. Different subtypes include:

  • Cutaneous T-cell Lymphoma (CTCL)
  • Sezary Syndrome

Leukaemic T-cell lymphoma

Leukemic T-cell lymphomas are found mainly in the blood and bone marrow.  Different subtypes include:

  • Adult T-Cell Leukaemia/Lymphoma (ATLL)
  • T-cell lymphoblastic lymphoma (LL)

Prognosis of peripheral T-cell lymphoma (PTCL)

The current standard first-line treatment for peripheral T-cell lymphoma (PTCL) is chemotherapy.  Patients commonly achieve remission (no signs of cancer detected) after first-line treatment.  Yet a high percentage of patients can relapse (the lymphoma returns) soon after.  There are currently research studies for peripheral T-cell lymphoma investigating ways to treat and improve outcomes for patients with this subtype of lymphoma.

Treatment for peripheral T-cell lymphoma (PTCL)

Once all of 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
  • Age
  • Past medical history & general health
  • Current physical and mental wellbeing
  • Patient preferences

The doctor and/or cancer nurse should explain the treatment plan to the patient and the possible side-effects. 

The standard first-line treatment for PTCL can include:

  • CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone)
  • CHOEP (CHOP with addition of etoposide)
  • Chemotherapy followed by autologous stem cell transplant (ASCT)
  • Radiotherapy
  • Clinical trial participation

There are many treatments that are being tested in clinical trials in Australia and around the world for PTCL.  Some of these treatments include:

  • Romidepsin (Istodax)
  • Pralatrexate (FOLOTYN)
  • Brentuximab Vedotin (Adcetris)
  • Pembrolizumab (Keytruda)
  • Nivolumab (Opdivo
  • Durvalumab (IMFINZI)
  • Lenalidomide (Revlimid)
  • Vorinostat (Zolinza)
  • Belinostat (Beleodaq)
  • Alemtuzumab
  • Bortezomib (velcade)
  • Carfilzomid (Kyprolis)
  • Temsirolimus
  • Deoxycoformycin (Nipent)
  • Tipifarnib (Zarnestra)
  • 5-Azacitidine (Vidaza)

Peripheral T-cell lymphoma Fact Sheet

For more info see
Understanding Clinical Trials

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