Each year, only about a thousand people are diagnosed with large granular lymphocyte leukemia (LGLL). A rare disease like this presents challenges because there’s no standard treatment protocol. However, doctors do have a range of options to control symptoms and help you return to a more normal life.
When Is LGL Leukemia Treatment Needed?
As awareness of the disease grows, many people may learn they have LGL leukemia by accident when they receive an abnormal blood test result. LGLL is a chronic disease, and it doesn’t always require treatment. About 50 percent of people with the disease will need treatment at some point, but many people will be “watch and wait.” This means your symptoms aren’t life-threatening or lowering your quality of life enough to justify undergoing treatment (PDF).
When you have certain symptoms, your doctor may recommend LGL leukemia treatment. Those symptoms include:
- Severe neutropenia, a neutrophil count below 500
- Neutropenic patients with frequent infections
- Symptomatic and/or transfusion-dependent anemia
- An autoimmune disorder, such as rheumatoid arthritis, that needs treatment
How Is LGL Leukemia Treated?
Many people with LGL leukemia also have an autoimmune disorder, and both conditions are treated similarly. The director of UVA Cancer Center, Dr. Thomas Loughran, Jr., and other experts in LGL leukemia recommend starting with oral methotrexate, an immunosuppressive drug, or cyclophosphamide (Cytoxan), an alkylating agent (affects DNA).
In a podcast interview, Dr. Loughran explained that in LGL leukemia, your immune system is too powerful. The cancer cells are making inflammatory cytokines, essentially poisons to your body, that attack bone marrow or joints. While inflammatory cytokines are part of our normal immune response to pathogens, in LGL leukemia this response does not get turned off. Methotrexate works to suppress your immune system and stop the production of those poisons. This drug may be the first choice for people with rheumatoid arthritis, neutropenia or another autoimmune disorder.
Cyclophosphamide works a little differently. LGL cells have clonal activity and make copies of themselves. This oral chemotherapy medication interrupts the cell division process and kills cancer cells. A low dose of cyclophosphamide for six to 12 months may be the first choice for people with anemia or pure red cell aplasia.
Along with taking a medication, your doctor will likely prescribe a healthy lifestyle as well. Exercise and balanced eating help you get stronger and feel better.
When Treatment Doesn’t Work
Everyone responds differently to the disease and to treatments. Your doctor may perform a blood test about four months into treatment to check your progress. The goal is to have a neutrophil count above 500, circulating LGL cells in a normal range and no further need for transfusions. If your disease is in remission or improving, your doctor will leave you on the first treatment for several more months, until she determines it’s safe to end treatment.
If your first treatment choice isn’t working, the next step is to choose an alternative first-line choice and perform another blood test in another four months. If both methotrexate and cyclophosphamide don’t work, your next option is the immunomodulatory (decreases the activity of immune cells) drug cyclosporine. In the vast majority of people needing treatment, one of these three options works.
In a small number of people, however, these three don’t work or the disease comes back. Alemtuzumab (Campath) is the next option. It is used to treat B-cell chronic lymphocytic leukemia and has shown promising results in LGLL patients as well.
It may take more than one try to find the best treatment option for you. Once you find one that works, you will likely be able to get back to a normal life without the symptoms you were experiencing before. You can be as physically active as you feel comfortable.
The Future of LGL Leukemia Treatment
Rare diseases present challenges in research because such small numbers of people are affected. However, researchers at UVA Cancer Center and around the world participate in clinical trials and research efforts to better understand the disease and possible treatments.
If you’ve been diagnosed with LGL leukemia, you can help aid that understanding by donating blood and tissue samples to the LGL Leukemia Registry. You can donate to the registry from anywhere by simply filling out some forms and working with your doctor to get the samples. These samples are transferred directly to the Loughran research laboratory where they are utilized to characterize the abnormal survival pathways in leukemic cells and to test the sensitivity to FDA-approved or experimental therapeutics. Therefore, the registry is one of the primary tools doctors and researchers have right now to discover and advance treatment options and improve the lives of people with this disease.
LGL Leukemia Program
The UVA Cancer Center has a state-of-the-art LGL Leukemia Program that offers you the best treatment possible. Learn More
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Hello
I came across your article above on LGLL and found it very educational, relate-able and easy to understand at a patient’s level. It is always hard to grasp the the why or how with this disease but I found this information helpful. I had LGLL 2 times, once in 2006 (age 40) and then in 2015. I was treated first at Stanford and then UCSF. Through follow up testing it appears it may be coming back so I may be in for another round of testing and treatment.
Thank you,
Jim
Hi Jim,
I’m sorry to hear that it has come back. My wife has been battling it foe the last 3 years. We are in LA, would there be any way to share with us your doctor’s contact information?
Sincerely,