Large Granular Lymphocytic Leukaemia

Large granular lymphocytic leukaemia (LGLL) is a type of chronic leukaemia, which affects the white blood cells known as T-cells.

Blood cells are formed in the bone marrow, which is the spongy tissue found inside the bones. Blood-forming stem cells divide to produce either more stem cells or immature cells that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell.

Large granular lymphocytic leukaemia (LGLL) affects T lymphocytes or, less commonly, NK lymphocytes.

What causes LGLL?

The cause(s) of LGLL are not known but it is important to understand that:

  • Leukaemia is not a condition which can be caught from someone else (contagious)
  • Leukaemia is not passed on from a parent to a child (inherited)

Risk factors:

  • Age – like most forms of cancer, LGLL is more common in older people, with only about 1 in 5 patients being younger than 50 years old when diagnosed
  • Genes – a gene called STAT-3 is abnormal in many patients with LGLL. It is not clear whether this is a cause of LGLL
  • Environment – LGLL is often seen in association with autoimmune disorders, such as rheumatoid arthritis. This suggests that chronic over activity of the immune system may be part of the cause of LGLL

Signs and symptoms of LGLL

The most common symptom at diagnosis is repeated infections, caused by low numbers of neutrophils (white cells which defend the body against infection). Even when the neutrophil count is very low, infections are rarely severe enough to be life-threatening.

Other common symptoms are anaemia, leading to tiredness and shortness of breath, and autoimmune conditions such as rheumatoid arthritis.

Between a quarter and a half of all patients will have an enlarged spleen, which can cause abdominal discomfort (tummy ache), especially after eating. Unlike some forms of leukaemia the liver and the lymph nodes (glands) are not usually enlarged.

Diagnosis

The most important test for diagnosis of LGLL is a full blood count. This will show the enlarged granular lymphocytes which give the disease its name. The blood count is likely to be repeated to check for progression of the disease and for response to treatment.

Other tests which may be done include:

  • X-rays, ultrasound or scans (CT or MRI) – To monitor impact on organs of the body

Treatment of LGLL

About half of all patients who have LGLL do not need treatment. Treatment is usually given if patients have symptoms such as repeated infections or anaemia.

LGLL is not considered curable but it usually responds well to treatment, when this is needed. Most patients with LGLL have a long survival with a good quality of life.

The main ways in which LGLL is treated are:

  • Immunosuppression – drugs which reduce the activity of the immune system – Most of the symptoms of LGLL are caused by over activity of the immune system
  • Purine analogue inhibitors (fludarabine, cladribine and pentostatin) – these help to reduce the number of lymphocytes, including the abnormal lymphocytes seen in LGLL
  • Surgery/splenectomy – If drug treatment does not help and the spleen is very enlarged an operation to remove the spleen (splenectomy) may help

The drugs used for LGLL are not cell-killing drugs, like those to treat many forms of cancer. This means that side effects are usually milder and do not include hair loss or nausea.

The details of your treatment will vary depending on the features of your LGLL and your general fitness. You will be given a chance to discuss treatment options and detailed information on your treatment plan before it starts. The side effects of treatment vary between different types of treatment and different patients. You will be given detailed information about any likely side effects before you start treatment.

Questions to ask your medical team about LGLL

We understand going through a blood cancer through journey can be difficult. It may help to talk to a close friend or relative about how you are feeling. Here are some questions that may be useful to ask your doctor.

  • How would I know if I had LGLL?
  • What tests will I need to have?
  • What will the tests show?
  • How long will the results take?
  • How rare is LGLL?
  • What sort of treatment will I need?
  • How long will my treatment last?
  • What will the side effects be?
  • Is there anything I should or shouldn’t eat?
  • Will I be able to go back to work?
  • Where can I get help with claiming benefits and grants?
  • Where can I get help dealing with my feelings?

Published date: August 2016

Review  date: August 2018