Hepatosplenic gamma delta T-cell lymphoma is a cancer of the lymphatic system and develops from T lymphocytes (white blood cells that fight infection). This type of lymphoma is a very rare, aggressive (fast growing) and can be difficult to treat.
Overview of hepatosplenic gamma delta T-cell lymphoma
Hepatosplenic gamma delta T-cell lymphoma is a very rare aggressive (fast growing) T-cell non-Hodgkin lymphoma (NHL). This lymphoma can be found in the spleen, liver, blood and bone marrow. It is known as an ‘extra nodal’ lymphoma (found outside of the lymph nodes).
Hepatosplenic gamma delta T-cell lymphoma is often grouped under the umbrella term peripheral T-cell lymphoma (PTCL). These are a group of aggressive T-cell non-Hodgkin lymphomas. They account for 7% of all NHL cases and 10% of all lymphoma cases.
Hepatosplenic gamma delta T-cell lymphoma (HSGDTCL) is named as:
- Hepato: means ‘relating to the liver’
- Splenic: means ‘relating to the spleen’
Hepatosplenic gamma delta T-cell lymphoma is a very rare and difficult to treat. The treatment for this subtype is chemotherapy. Many people relapse (lymphoma comes back) after first line treatment. If remission (no signs of cancer) is achieved, an allogeneic stem cell transplant is often recommended for a chance of cure (not suitable for everyone).
Who is affected by hepatosplenic gamma delta T-cell lymphoma
Hepatosplenic gamma delta T-cell lymphoma often occurs in young adults, where the average age of diagnosis is around 30 years of age. This subtype is more common in males.
Individuals who are at a higher risk of developing hepatosplenic gamma delta T-cell lymphoma may include:
- History of inflammatory bowel disease, particularly if on systemic immunosuppressive therapy
- History of ongoing use of immunosuppression (solid organ transplant or bone marrow transplant)
Symptoms of hepatosplenic gamma delta T-cell lymphoma
Hepatosplenic gamma delta T-cell lymphoma is an aggressive lymphoma, meaning it is usually fast growing. Symptoms usually have a fast onset of usually weeks for symptoms to present.
The most common first symptoms of hepatosplenic gamma delta T-cell lymphoma is feeling bloated or discomfort in your abdomen (stomach). This lymphoma does not usually cause swollen lymph glands like other lymphomas, as it is usually ‘extra nodal’ (found outside of the lymph nodes).
Other symptoms may include:
- Swollen liver, which might make you feel bloated, cause fluid build- up in your tummy. Or make your whites of your eyes yellow (jaundice)
- Swollen spleen, which might cause pain behind your ribs on the left side, or make you feel full very quickly when you are eating
- Anaemia (low red blood cell count)
- Thrombocytopenia (low platelets)
- Neutropenia (low neutrophil count)
B symptoms are what doctors call the following symptoms and can include:
- Persistent fevers (especially at night >38C),
- Unexplained weight loss
- Night sweats (that drench sleepwear & bedding)
Diagnosis of hepatosplenic gamma delta T-cell lymphoma
A biopsy is always required for a diagnosis of hepatosplenic gamma delta T-cell lymphoma. A biopsy is a surgical procedure to remove part abnormal tissue. The pathologist will then look at it under the microscope to see what the cells look like. The biopsy can be done under local or general anaesthetic depending on what part of the body is being biopsied.
Hepatosplenic gamma delta T-cell lymphoma can often be difficult to diagnose. A biopsy of the liver or the spleen is needed for a diagnosis.
Waiting for results can be a difficult time. It may help to talk to your family, friends or a specialist cancer nurse.
Staging of hepatosplenic gamma delta T-cell lymphoma
Once a diagnosis of hepatosplenic gamma delta T-cell lymphoma is made, further tests are required to see where else in the body the lymphoma has affected or located. This is called staging. The staging of the lymphoma helps the doctor determine the best treatment for the patient.
Staging scans and tests
The scans and tests needed for staging before and after treatment can start may include:
- Positron emission tomography (Pet) scan
- Computed tomography (CT) scan
- Bone marrow biopsy
- Further blood tests
Patients may 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 of the necessary biopsies, tests and scans 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.
Prognosis of hepatosplenic gamma delta T-cell lymphoma
Hepatosplenic gamma delta T-cell lymphoma is very rare and difficult to treat. First line treatment for this subtype is chemotherapy. Many people relapse (lymphoma comes back) after first line treatment. If remission (no signs of cancer) is achieved, an allogeneic stem cell transplant may be recommended for a chance of cure (not suitable for everyone).
Treatment of hepatosplenic gamma delta T-cell lymphoma
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
- 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. Hepatosplenic gamma delta T-cell lymphoma is difficult to treat, and it is common for this lymphoma to relapse (come back).
The standard first line chemotherapy regimens used may include:
- CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone)
- CHOEP (CHOP with the addition of etoposide)
- ICE (ifosfamide, carboplatin and etoposide)
- IVAC (ifosfamide, etoposide and cytarabine)
- Clinical trial participation
- Chemotherapy followed by an allogeneic stem cell transplant (donor stem cells – preferred)
- Chemotherapy followed by an autologous stem cell transplant (own stem cells)
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)
- Thrombocytopaenia (low platelets that help bleeding and clotting)
- Neutropenia (low white blood cells help with immunity)
- Nausea and vomiting
- Bowel problems such as constipation or diarrhea
- Fatigue (tiredness or lack of energy)
- Reduced fertility
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 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 this is more likely with certain chemotherapy protocols (combinations of drugs) and high-dose chemotherapy used before a stem cell transplant. Radiotherapy to the pelvis also increases the likelihood of reduced fertility. Some antibody therapies may also affect fertility, but this is less clear.
The doctor should advise on whether fertility may be affected or whether fertility preservation should be done before the treatment has started. Patients should always ask about fertility preservation before treatments starts if this is important to them.
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.
Relapse of refractory management of hepatosplenic gamma delta T-cell lymphoma
For some people, initial treatment is effective, and the lymphoma does not return after treatment. However, for some people 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.
The second-line or third line treatments may include:
- 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 for hepatosplenic gamma delta T-cell lymphoma
There are many treatments that are currently being tested in clinical trials around the world for patients with both newly diagnosed and relapsed lymphoma. For a list of the current treatments that are being investigated for hepatosplenic gamma delta T-cell lymphoma include:
- Monoclonal antibodies
- Proteasome inhibitors
- Histone deacetylase inhibitor (HDAC)
Peripheral T-cell lymphoma (PTCL) subtypes
For more information on different subtypes see below:
- Peripheral T-cell lymphoma – not otherwise specified (PTCL-NOS)
- Angioimmunoblastic T-cell lymphoma (AITL)
- Anaplastic large cell lymphoma (ALCL)
- 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 (CTCL)
- Sezary Syndrome
- Adult T-Cell Leukaemia/Lymphoma (ATLL)
- T-cell lymphoblastic lymphoma (LL)
What happens after treatment?
Finishing treatment can be a challenging time for many people, as they readjust back into life after treatment. Some people may not start to feel some of these challenges for weeks to months after cancer treatment has completed, as they start to reflect on their experience or do not feel that they are recovering has completed, as they start to reflect on their experience or do not feel that they are recovering as quickly as they should. 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.