Hepatosplenic T-cell lymphoma

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What is hepatosplenic T-cell lymphoma and who gets it?

Hepatosplenic T-cell lymphoma is a very rare type of non-Hodgkin lymphoma (NHL) that develops from T lymphocytes (T cells). Our overview page explains what T-cell lymphoma is.

Hepatosplenic T-cell lymphoma causes swelling of the liver and spleen:

  • ‘hepato’ refers to the liver
  • ‘splenic’ refers to the spleen.

Hepatosplenic T-cell lymphoma usually occurs in younger adults, with an average age in the mid-30s at the time of diagnosis. It can occur in children. It affects more men than women.

Hepatosplenic T-cell lymphoma is sometimes linked with immunosuppression (lowered immunity), for example, it can occur after organ transplant or after treatment for inflammatory bowel conditions like Crohn’s disease. However, it is very rare for people with these conditions to develop hepatosplenic T-cell lymphoma.

What are the symptoms of hepatosplenic T-cell lymphoma?

Hepatosplenic T-cell lymphoma causes a range of symptoms. Most people have:

  • enlarged (swollen) spleen and liver
  • anaemia (low red blood counts), which can make you tired and short of breath
  • thrombocytopenia (low platelets), which can make you more likely to bruise and bleed; low platelets are particularly noticeable in this type of lymphoma
  • neutropenia (low neutrophils), which can increase your risk of infection
  • B symptoms’ (fevers, night sweats and weight loss), which often appear together.

You might also have enlarged lymph nodes (glands), but enlarged liver and spleen are more common.

Abnormal T cells are usually found in the bone marrow and sometimes in the blood.

The vast majority of people have advanced disease when they are diagnosed.

How is hepatosplenic T-cell lymphoma treated?

T-cell lymphomas are rare, so it is difficult to determine which treatment gives the best outcome. You might be asked if you would like to take part in a clinical trial to help test new and current treatments.

Hepatosplenic T-cell lymphoma is usually difficult to treat. It is common for the lymphoma to relapse (come back).

You are most likely to be treated with a chemotherapy regimen (combination of drugs), eg CHOP, which is made up of:

  • cyclophosphamide
  • doxorubicin (hydroxydaunorubicin
  • vincristine (Oncovin®)
  • prednisolone (a steroid).

Your doctor might suggest a more intensive chemotherapy regimen if you are well enough. A chemotherapy drug called pentostatin is sometimes used.

Chemotherapy is given in cycles. The drugs are given on certain days, which are followed by a break in treatment for your body to recover before the next cycle begins.

If you are well enough, and your lymphoma has been reduced by chemotherapy, your doctor is likely to recommend you have more chemotherapy together with a stem cell transplant. If a suitable donor is available, this might be an allogeneic (donor) stem cell transplant. An allogeneic stem cell transplant is a very intensive form of treatment but can sometimes lead to a long-term remission (no evidence of lymphoma).

What happens if hepatosplenic T-cell lymphoma comes back or doesn’t respond to treatment?

It is common for hepatosplenic T-cell lymphoma to relapse after being in remission. Sometimes, the lymphoma is refractory (doesn’t respond) to treatment. In these cases, your doctor might consider:

  • a different chemotherapy regimen
  • an allogeneic (donor) stem cell transplant if your lymphoma is reduced by chemotherapy and you are well enough
  • a newer drug, usually through a clinical trial.

Hepatosplenic T-cell lymphoma is very rare, so suitable clinical trials are likely to be recruiting people with several types of T-cell lymphoma. Your general health is also an important factor in whether or not you can enter a trial. Clinical trials only recruit people who are well enough to tolerate the study treatment. Your doctor can advise you whether there is a trial suitable for you.

What happens during follow-up?

When your treatment is finished, you have regular follow-up appointments at the hospital. This follow-up period is to:

  • see how you are feeling
  • check you have recovered from your treatment
  • check there are no signs of the lymphoma relapsing
  • look for any late effects (side effects that develop months or years after treatment) of your treatment.

How often you have appointments depends on your hospital’s practice and what treatment you’ve had. You are usually seen every 1–3 months at first. Your appointments then become less frequent if you are well over time. Your follow-up usually stops after around 3 years if you remain in remission. You should contact your medical team or GP if you develop any symptoms of lymphoma or have other concerns between your appointments. Your specialist might bring your appointment forward if they think they need to see you sooner.

Sources used

These are some of the sources we used to prepare this information. The full list of sources is available on request. Please contact us by email at publications@essais-cliniques-lymphomes.fr or phone on 01296 619409 if you would like a copy.

British Committee for Standards in Haematology (BCSH). Guidelines for the management of mature T-cell and NK-cell neoplasms (excluding cutaneous T-cell lymphoma). August 2013. Available at: www.bit.do/bsh-guidelines-tnhl (Accessed August 2016).

Kircher SM et al. Chapter 14: T-cell non-Hodgkin lymphoma. In Marcus R et al. Lymphoma: pathology, diagnosis and treatment. 2nd edition. Cambridge University Press. Published 2014.

Armitage JO. The aggressive peripheral T-cell lymphomas: 2015. American Journal of Hematology. 2015; 90: 665-73. Available at: www.bit.do/aggressive-tcell (Accessed August 2016).

Sims MC, et al. Long-term outcomes of autologous stem cell transplantation for peripheral T-cell lymphomas across the Thames Valley (1997–2012). British Journal of Haematology, 2015. 169: 452–455. Available at: www.bit.do/sct-tcell (Accessed August 2016).

Swerdlow SH, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood, 2016. 127: 2375–2390. Available at: www.bit.do/tcell-classification (Accessed August 2016).

Acknowledgements

With thanks to Dr Chris Hatton, Consultant Haematologist, Churchill Hospital, Oxford, for reviewing this information.

We would also like to thank the members of our Reader Panel who gave their time to review this information.

Content last reviewed: August 2016

Next planned review: August 2019

More information: 

If you would like a printed copy of any of our lymphoma information, complete our information order form and we will post it to you free of charge (UK, Channel Islands and Republic of Ireland only). If you live outside the UK, we recommend that you contact the lymphoma patient and carer organisation in your country as treatments and healthcare systems vary overseas.

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