Treatment of HSTCL

Hepatosplenic T-cell lymphoma is difficult to treat and it is common for the lymphoma to come back (relapse). Because it is rare, no treatments have become established as internationally recognised standards of care for hepatosplenic T-cell lymphoma. Clinical trials in this rare condition are unusual. 

However, some trials of experimental agents do allow people with hepatosplenic T-cell lymphoma to take part, particularly if their lymphoma has relapsed. Several targeted drugs are being tested. Your doctor might ask you if you would like to take part in a clinical trial to help test new treatments and to find out what the best treatment is for hepatosplenic T-cell lymphoma.

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 regimens used for hepatosplenic T-cell lymphoma are:

• CHOP: cyclophosphamide, doxorubicin (or hydroxydaunorubicin), vincristine (also known as Oncovin®) and prednisolone
• CHEOP: CHOP plus etoposide
• ICE: ifosfamide, carboplatin and etoposide
• IVAC: ifosfamide, etoposide (also known as VP-16) and cytarabine (also known as Ara-C).

However, your medical team might recommend a different chemotherapy regimen.

If you respond to chemotherapy and you are well enough, your doctor might recommend that you have a stem cell transplant. This could be a donor (allogeneic) stem cell transplant or a self (autologous) stem cell transplant. A stem cell transplant might give you a better chance of staying in remission (no evidence of lymphoma).

Below you will find clinical trials we have found in the US and abroad.  We continue our research to find countries who may offer other treatments and clinical trials.

The following clinical trials are still open as of January 28. 2022 and were were found on the T-Cell Leukemia Lymphoma foundations website:

• NCT01703949: Brentuximab Vedotin in Treating Patients with Relapsed or Refractory CD30+ Lymphoma
• NCT02168140: This trial studies the side effects and best dose of CPI-613 when given together with bendamustine hydrochloride in different conditions. 
• NCT02232516: The purpose of this study is to evaluate how safe and effective the combination of romidepsin and lenalidomide is for treating patients with PTCL.
• NCT02588651: This study will include patients with MTCL that has been treated with at least one type of chemotherapy, but is not responding.
• NCT03075553: This phase II trial studies how well nivolumab works in treating patients with peripheral T-cell lymphoma that has come back after a period of improvement or that does not respond to treatment.

Please check T-Cell Leukemia Lymphoma for current information in US-based clinical trials.

Also, please ask your doctor about clinical trials they may be aware of.

The following clinical trials are still open as of January 28, 2022 and were found on the Lymphoma Action site in the UK:

• TREATT: a phase 3 trial testing tranexamic acid to reduce bleeding after chemotherapy
• BOOST: studying samples from stem cell transplant donors and recipients to improve transplant success rates and reduce side effects
• ToTem: a phase 1 trial of donor ‘effector memory’ T cells to improve immune system recovery after donor (allogeneic) stem cell transplants
• A phase 1 trial of venetoclax in children and young adults with relapsed or refractory cancers, including non-Hodgkin lymphomas
• SGN35-032: A phase 2 trial of brentuximab vedotin plus chemotherapy in people with peripheral T-cell lymphoma
• CCS1477-02: a phase 1/2a study of CCS1477 in blood cancers, including relapsed or refractory non-Hodgkin lymphoma
• ECHELON-2: a phase 3 trial comparing brentuximab vedotin and CHP chemotherapy with standard CHOP in people with T-cell lymphoma that has not yet been treated

Please check Lymphoma Action for current information on clinical trials.

Many targeted drugs are being tested to see if they can help people with T-cell lymphoma. Drugs that are being tested in hepatosplenic T-cell lymphoma include:

• antibody treatments such as alemtuzumab, which binds to a protein called CD52 on T cells
• proteasome inhibitors such carfilzomib
• HDAC inhibitors such as romidepsin.