Large Granular Lymphocyte Leukemia (LGL)

Large granular lymphocyte leukemia is chronic lymphocytic leukemia. It has two types:

• T-cell large granular lymphocytic leukemia (T-LGL).
• Chronic lymphoproliferative disorder of NK-cells (CLPD-NK), also known as chronic NK cell lymphocytosis and aggressive NK cell leukemia.

Both kinds of LGL leukemia start in white blood cells known as cytotoxic T-cells or natural killer (NK) cells. These cells change (mutate) and keep your bone marrow from making healthy blood cells. This leukemia usually grows slowly. It can go away but can also come back and become a chronic health condition.

You may never have symptoms and find out you have LGL when you get routine blood tests as part of a physical exam. If you do have symptoms, you might feel tired all the time, get frequent fevers and infections or have an enlarged spleen (splenomegaly). If you have LGL, you’re also at risk of autoimmune conditions like rheumatoid arthritis.

But no matter what symptoms you have (or don’t have), you’ll want to make sure you’re working with healthcare providers who are familiar with this rare leukemia.

What to expect at your first visit

When you come to Cleveland Clinic, you can be confident that you’re working with a team experienced in diagnosing and treating large granular lymphocyte leukemia. We know what it takes to confirm a diagnosis and get you on the best treatment path with the answers, support and guidance you need.

The first step in making this happen is an important one — sharing your story. Your provider will want to know all about how you’ve been feeling, in your own words. They’ll ask you to share:

• Your symptoms, if any.
• How long you’ve had these symptoms.
• If your symptoms are getting worse.
• How you’ve been feeling.
• If you’ve had tests before this appointment.
• Any other health conditions you may have.
• If family members have had any cancers or serious health conditions.

Your provider will give you a physical exam to check your overall health and look for signs of LGL leukemia. They’ll also order some tests to help rule out other conditions or pinpoint a diagnosis.

Testing for large granular lymphocyte leukemia

To confirm a diagnosis (and type) of large granular lymphocyte leukemia, we’ll need to do some more tests. We can learn a lot about what’s going on from the results of tests like:

• Complete blood count (CBC) with differential: Measures the number of each type of blood cell.
• Peripheral blood smear: Tests for abnormal blood cells.
• Bone marrow aspiration and biopsy: Looks for abnormal cells in your bone marrow’s liquid and in the spongy bone tissue.
• Flow cytometry: Uses lasers to quickly and precisely analyze the characteristics of cells.
• Genetic testing: Identifies genetic mutations (changes) that could point to an LGL leukemia diagnosis.
• T-cell receptor (TCR) gene rearrangement analysis: Tests blood or bone marrow for genetic problems that affect how T-cells work.

Getting a second opinion for large granular lymphocyte leukemia

When you learn you may have rare leukemia, you might feel emotional, overwhelmed or even numb. But you probably also know you want the best possible care every step of the way — from healthcare providers you trust. That’s why we encourage second opinions.

We know that cancer doesn’t wait, so we always try to meet as soon as possible. Our expert blood cancer specialists will dive in to help you understand your diagnosis and available treatment options. Getting a second opinion can help you feel more in control of your care. And give you peace of mind knowing you’re in the most capable hands.

Meet Our Large Granular Lymphocyte Leukemia Team

Large granular lymphocyte leukemia affects people in different ways. Cleveland Clinic’s team-based approach to care means you get all the providers you need, no matter what specialties, on one personalized care team. Our hematologists and oncologists lead this team, which could also include:

• Pathologists.
• Rheumatologists.
• Nurse practitioners.
• Palliative care.
• Genetic counselors.
• Psychologists.
• Physician assistants.
• Care coordinators (providers who keep you up to date on your care plan and advocate on your behalf).
• Social workers.

Your care team stays by your side throughout your cancer journey. These providers meet weekly to talk about your care plan, progress and next steps in treatment. We work together to make sure you’re getting the care that’s best for you.

Treatment for LGL leukemia is different from person to person. It depends on the type you have and if you have symptoms.

Many people with this condition start with “watchful waiting.” We delay treatment, keep a close eye on your symptoms and repeat blood tests over time. And if symptoms come up or we notice changes in your test results, we can start treatment quickly.

When your symptoms or tests show the condition is progressing, we may recommend any combination of treatments depending on your needs, like:

• Corticosteroids: Help your bone marrow make healthy cells.
• Immunosuppressive medications: Help keep your immune system in check to slow or stop abnormal cell growth.
• Blood transfusion: Infuses healthy donated blood into your system to reduce symptoms like fatigue.
• G-CSF (granulocyte-colony stimulating factor): Helps increase infection-fighting white blood cells called neutrophils.

We carefully monitor your progress throughout treatment. This may include repeating tests to see if the treatments are effective. If LGL leukemia becomes advanced or comes back, we may recommend:

• Alemtuzumab: New targeted therapy that destroys certain types of cells made by your immune system.
• Clinical trials: Study the latest potential treatments.
• Splenectomy: Surgical removal of your spleen.
• Stem cell transplant: Replaces abnormal stem cells that make blood cells with healthy donor cells.

Living With Large Granular Lymphocytic Leukemia

When you have LGL leukemia, there’s a good chance it may come and go. So, it’s important to stay in touch with your care team through regular follow-up visits and testing. And to stay in tune with your body by keeping an eye out for new symptoms or symptoms that get worse.

We understand that living with a chronic condition can feel like a lot to handle at times. We offer support groups to connect you with other people  and families living with leukemia. Or we can help you schedule an appointment with our psychologists for counseling (psychotherapy). Your emotional and mental well-being is just as important as your physical health.

Taking the Next Step

Life with chronic large granular lymphocyte leukemia can be clouded by fatigue, uncertainty and fear of the unknown. Cleveland Clinic’s healthcare providers combine expert treatment with compassionate care and support across your entire journey. We constantly check up on your health. And, if and when you need treatment, we offer every available option to relieve symptoms — and even possibly put your LGL leukemia in remission — so you can keep enjoying all that life offers.