What is chronic large granular lymphocytic leukaemia (LGLL)?

Chronic LGLL is a subtype of the rare blood cancer LGLL. Within these webpages you will find out what it is and how it is diagnosed.

Large granular lymphocytic leukaemia (LGLL) is a rare blood cancer. It is a cancer of the white blood cells in your blood called lymphocytes. Chronic LGLL is one of the subtypes of LGLL. It occurs often in patients over 60 years of age.

The types of lymphocytes commonly increased in LGLL are:

  • T-lymphocytes (T-cells)
  • Natural killer lymphocytes (NK-cells)

There are three subtypes of LGLL

  • T-cell LGLL occurring in 85% of patients
  • NK-cell LGLL occurring in 10% of patients
  • Aggressive NK-cell LGLL occurring in 5% of LGLL patients

What are the signs and symptoms of chronic LGLL?

If you have chronic LGLL, you may have any of the following symptoms:

  • Persistent fatigue
  • Pale appearance, dizziness, heart flutters and shortness of breath on exercising
  • Recurring infections
  • Easy bleeding/bruising
  • Enlarged spleen
  • Weight loss
  • Fever
  • Night sweats

It is possible to have no symptoms at diagnosis. Symptoms will generally appear and progress slowly in chronic LGLL.

It may be an incidental finding (blood test performed for an unrelated reason).

If you have any of the above symptoms, contact your GP and ask for a blood test.

How is chronic LGLL diagnosed?

To diagnose LGLL, your healthcare team will perform blood tests. The reason behind the blood test is because a diagnosis of LGLL can only be made:

  • If you have had LGLL cells in your blood for more than six months. The increased numbers of LGLL cells should be greater than 0.5×109/L (normal range is 0.25×109/L).

As well as the above, you should have:

  • LGLL symptoms as well as increased LGLL cells present in your blood
  • Distinct cluster of differentiation (CD) proteins on T-cell LGLL and NK-cell LGLL cells. Immunophenotyping can display these CDs found on the surface of white blood cells
    • T-cell LGLL cells usually have CD3/CD8/CD57 proteins
    • NK-cell LGLL cells usually have CD2/CD16/CD56 proteins, but not the CD3 protein
  • The LGLL cells all look the same and therefore come from the same abnormal population of LGLL cells. This is known as “clonality”.
  • LGLL cells may show any of the following genetic mutations:
  • STAT3
  • STAT5B
  • TNFAIP3

If the results of the blood smear are unclear, a bone marrow aspiration/biopsy can be used to confirm the diagnosis.

Want to know more about LGLL?

You can find more information about LGLL in our free information booklet, download your copy here. If you’d rather read the booklet in smaller bitesize pieces of information, download our LGLL factsheet here.

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    Page published on: 15th August 2022