Treatment options for large granular lymphocytic leukaemia (LGLL)

If you have no symptoms, or only minor symptoms at diagnosis, you will likely not receive treatment. This is called active monitoring. It is sometimes known as watch and wait. Approximately 40% of LGLL patients have active monitoring.

When you do need treatment, your consultant will recommend when to start treatment.

View our Watch and Wait (also known as Active Monitoring) booklet here.

You should still have regular check up appointments. If you have questions about your care during active monitoring, you can speak to our advocacy team for more information by emailing advocacy@leukaemiacare.org.uk.

There are three main types of treatments available for LGLL treatment. They are:

• Immunosuppressants
• Immunotherapy
• Chemotherapy combinations

Splenectomy is removal of the spleen. It is a treatment option if you have an enlarged spleen which causes symptoms.

First line treatment for LGLL is immunosuppressants. These consist of methotrexate, cyclophosphamide and cyclosporin A.

• Steroids, such as prednisone, are useful when used in combination with immunosuppressants. They are of limited value on their own.
• A period of at least four months must elapse before assessing the patients’ response to treatment of T-LGLL and NK-LGLL.

If you would like to understand more about the order of treatment for LGLL, you can read our LGLL booklet here.

Immunotherapy uses the body’s own immune system to fight the leukaemia cells. Immunotherapy can help patients:

• Who respond to treatment, but then the response stops (relapse or recurrence).
• Where treatment has not worked.
• Who show progression of the LGLL within six months of the last treatment dose (refractory LGLL).

Monoclonal antibodies are effective types of immunotherapy in LGLL. They act on specific receptors on the LGLL cells.

• Alemtuzumab is a monoclonal antibody which targets leukaemia cells with CD52 proteins.
• Rituximab is a monoclonal antibody targeting the CD20 protein. This has shown efficacy in treating T-LGLL in a small number patients also with rheumatoid arthritis.

Please note: these are not standard treatments and the results are based on small studies.

For the few patients with LGLL do not respond to immunosuppressants, combinations of chemotherapies may be helpful. These chemotherapies include fludarabine, cladribine, and bendamustine. There are very little trials of these chemotherapy combinations.

Splenectomy or removal of the spleen is a supplementary treatment option. It occurs if you have:

• An enlarged spleen which is causing symptoms
• Low levels of blood cells

The ten-year overall survival of all patients with chronic LGLL is 78%. Deaths, which occur in less than 10% of patients, are due to severe infections.
Factors which increase survival in patients with chronic LGLL are:

• Receiving an early diagnosis
• Prompt treatment