LGL Leukemia: FAQs

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Every patient or caregiver has their own unique experience with LGL leukemia. However, there are many questions that we hear frequently regarding symptoms, treatments, and daily concerns on living with LGLL. In this FAQ section, we will attempt to cover the most common questions that we receive.  New questions and answers will be posted periodically, so please check back for more information. 

Have a question that you would like answered? Submit it to LGLQuestion@virginia.edu, and it may be featured with an upcoming addition.

Coronavirus & LGL Leukemia FAQs

What is known about T-cell immunity and COVID-19?

Data shows the importance of T-cell immunity, as this type of lymphocyte controls the coronavirus infection.

One study shows that patients not exposed to this virus may already have some degree of cellular immunity. The theory is that this immunity is due to previous exposure to other coronavirus infections, like the ones that cause the common cold. 

Are there special precautions LGL leukemia patients should take?

LGL leukemia patients should take the same precautions and practice the same safety guidelines as everyone else. 

Most importantly, talk to your doctor if you have questions about your specific situation.

Should LGL leukemia patients get the COVID-19 vaccine?

Yes, LGL leukemia patients should get the COVID-19 vaccine. The vaccines are effective and have passed through strict clinical trial safety criteria in thousands of patients. Talk to your doctor about any questions or concerns.

For more information, see the general COVID-19 FAQs.

I've heard that the vaccine is less effective in people with blood cancer. Should I be concerned?

No. LGL leukemia doesn't reduce the kinds of blood cells that work with the vaccine. In fact, we've seen reports of vaccinated LGL leukemia patients who have responded well to the vaccine.

But, you may be taking medicine that lowers your immune system. These immunosuppressive medicines do reduce the kinds of blood cells that build your immunity.

In that case, if you're already vaccinated, your doctor can test your immune response. They may have concerns about your level of immunity. The test will show what that level is. You may need an additional shot of the vaccine to build the right level of immunity.

If you aren't vaccinated, talk to your doctor about it. They may recommend pausing your immunosuppressive medicines if it's safe. This lets your immune system work with the vaccine without the medicine interfering.

What is antibody testing for? How does it relate to the COVID vaccine?

We use antibody testing (called "SARS-CoV-2 Serology COVID-19 with spike protein") to see how your body has responded to the vaccine.

A positive antibody test result means your body used the vaccine to create antibodies to the coronavirus. Specific test results should always be discussed with your doctor.


General LGL leukemia FAQs

Are there any lifestyle changes I should consider after my diagnosis of LGL leukemia?

We always recommend you consult your primary care doctor and oncologist prior to making any lifestyle changes. Since LGL leukemia is a chronic disease, maintaining a healthy lifestyle can ensure that you feel your best. Talk to your doctor about:

  • How to eat a healthy balanced diet
  • How to exercise regularly
  • If you would benefit from a supplement

You should also use alcohol in moderation and avoid tobacco.  

Are there different stages of LGL leukemia?

No. We do not use stages or staging protocol for LGL leukemia.

Generally, we label cancers in terms of a stage, usually from 1 to 4, to describe how far the cancer has progressed. We do not use stages for leukemias since, by definition, they've already spread throughout the body via the blood system. 

Should I take antibiotics prior to having dental work done?

There are general guidelines for use of antibiotics for dental work in certain circumstances that should be followed in all cases. These recommendations are primarily for people who have heart conditions such as a murmur or heart valve disease. 

Prescription of prophylactic antibiotics (taking antibiotics as a preventive measure) is done to prevent mouth bacteria from spreading to other parts of the body and causing an infection.

In LGL leukemia, it is not absolutely necessary to have antibiotics prescribed for a routine dental cleaning unless your ANC is persistently lower than 500. If your ANC is greater than 500 but lower than normal blood values, and you are planning to have extensive dental work, the use of prophylactic antibiotics could be considered. 

It is always best to seek the advice of your dentist for your unique situation.  

Why do I still get so many infections even though my blood work is normal? 

If your blood counts are normal, there is no indication that you are at an increased rate of infection due to LGL leukemia. LGL leukemia patients are more susceptible to infections if their absolute neutrophil count (ANC) is lower than normal. Your body uses neutrophils to fight off bacterial infections, therefore if there are fewer to begin with, it may be more difficult for your body to fight an infection.

With ANC levels that are persistently high (more than 500), LGL leukemia patients are safe from serious infection. With ANC levels under 500, there is a risk of infection. Patients with very severe neutropenia have an ANC below 100 and are at greatest risk of a severe infection.

Should I travel if my neutrophil count is low?

Generally, we consider travel safe. Infections that patients develop almost never originate from the environment or from someone else who is sick. When the ANC is low, patients may become susceptible to infections that result from the bacteria (germs) that normally live in our bodies. The best advice is to wash hands frequently. If you must travel for an extended time on an airplane, consider using a mask while the air recirculates. 

Is it safe to receive vaccinations?

There really are no data on risks of vaccination for LGL leukemia patients. However, to be extra safe, we advise that you avoid live virus vaccinations. This is general advice for patients with a compromised immune system. 

Is it safe for a family member living with me to receive vaccinations?

It should be safe for a family member living with the patient to receive vaccines, even those that are live virus.

If I have a fever, should I go to the ER?

Our general advice is that if you have a persistent fever for more than a few hours of 101 or above, you should be seen in the ER. If you have an ANC below 500 with fever at that time, it is most likely that you will be admitted to the hospital for IV antibiotics, just to be safe.

Is there a relationship between LGL leukemia and autoimmune disease?

One of the goals of our research is to try and understand if there is a link between LGL leukemia and autoimmune disease. T-LGL leukemia is often associated with the autoimmune disease rheumatoid arthritis. Research has shown that autoimmune disease is often present in the family history of LGL leukemia patients. The “which came first” and whether one causes the other are not known and are being investigated.

Given the treatment regimens and chronic nature of LGL leukemia, why is it classified as a cancer and not an autoimmune disease? 

Although LGL leukemia is chronic and managed with immunosuppressant treatment, as in autoimmune disease, it is defined as a cancer because there is clonal expansion (the cells are copying themselves) of either T cells or NK cells. For the T-cell type, a TCR rearrangement test will confirm the clonal expansion. If the clonality is not present, then it is not officially LGL leukemia. In the NK-cell type, it is harder to show clonality, but it could be established if chromosomal abnormalities are present. The disease was named leukemia because the cells are clonal and are in the blood.

Are Neupogen or Neulasta recommended as primary treatment options for LGL leukemia patients?

No. Right now, immunosuppressive treatment is the standard treatment for LGL leukemia patients with neutropenia (low neutrophil count) symptoms. But, in cases where your neutrophil count needs to be raised quickly, Neupogen or Neulasta could be good options. These medicines do cause your bone marrow to produce more neutrophils, but they don't treat the underlying cause of your low neutrophil production.

I am constantly fighting fatigue. Is this a symptom of LGL leukemia or a symptom of the treatment for it?

This is a very difficult question to answer because there can be many reasons, other than LGL leukemia, to have fatigue. One reason to have fatigue that would be related to LGL leukemia would be the presence of anemia. That could be an indication for treatment of LGL leukemia. Interestingly, some LGL leukemia patients have noted a direct correlation between their fatigue and the results of their neutrophil count: the lower the neutrophil count, the more severe the symptoms of fatigue. This we don't usually think of fatigue this way, we've observed this in a number of our patients.

I have bone pain. Is this related to the LGL leukemia?

Bone pain would not be part of LGL leukemia. Sometimes our patients receive growth factors to help their neutrophil count increase (Neupogen, Neulasta). Bone pain is a common side effect of those medicines.

It appears that some people with LGL leukemia have symptoms while others do not. If I have had symptoms am I more likely to experience others in the future?

The problems that occur most frequently in patients with LGL leukemia are low blood counts or rheumatoid arthritis. Generally, symptoms that develop are related to these problems. If the neutrophil count is too low (neutropenia), then patients may suffer from repeated bacterial infections. If the red cell count is too low (anemia) then patients may experience fatigue or shortness of breath when they exert themselves. Patients with LGL and rheumatoid arthritis are subject to the arthritis symptoms that can include pain, stiffness, and swelling in multiple joints.

So, someone is not destined to continue to have problems in the future if treatment is successful.

Is LGL leukemia hereditary?

No, LGL leukemia is not inherited. Although patients may have mutations present in their leukemic LGL cells, these are acquired mutations and not present in other cells of the body.

Can biologics (for example: vaccines, blood and blood components, gene therapy, tissues) or environmental agents cause LGL leukemia?

While rare, development of T-LGL leukemia after solid organ or hematopoietic (blood) stem cell transplant can occur. Currently, we have no definite evidence to support that any environmental agent can cause LGL leukemia.  We continue to investigate the theory that the initial event leading to development of LGL leukemia may be infection with a new type of virus.

Is bone marrow transplant (BMT) or stem cell transplant (SCT) a good treatment option for LGL leukemia?

No, we don’t recommend BMT/SCT for most patients with LGL leukemia, because:

  • Most patients will respond to the usual medications for LGL leukemia and therefore not need a stem cell transplant
  • Stem cell transplants have a high chance of serious side effects, including death

In instances where the standard medicines for LGL leukemia have been unsuccessful, your oncologist may discuss BMT/SCT with you.

About BMT/SCT Therapy

Two types of BMT/SCT therapy exist:

  • Autologous uses a patient’s own bone marrow stem cells, which reduces the chance of the body rejecting the transplant.
  • Allogeneic uses a healthy donor’s bone marrow stem cells, which carries the risk of graft-versus-host-disease (the new immune system attacks the patient’s own body). However, the new immune system also attacks any residual leukemia, so there may be less chance of relapse. 

Research: BMT/SCT in LGL Leukemia

A recent clinical study of stem cell transplants in a limited number of T-LGL leukemia patients reported death in about 40% of patients receiving autologous or allogeneic transplant treatment. The patients who survived were still alive at the time of the report’s publication. However, the study used a very small sample size; more research is needed. 

Is immunotherapy or chimeric antigen receptor (CAR)-T cell therapy a good treatment option for LGL leukemia?

No, these are not good treatment options for LGL leukemia. 

Immunotherapy means taking a patient’s own immune cells and stimulating or modifying the cells, giving the cells new abilities. One example is removing a patient’s T-cells and activating them. Such an approach would not work in LGL leukemia, since the T-cells are already activated (“turned on”) and we would not want these cells to be turned on even more. Furthermore, current treatment of LGL leukemia is the opposite: immunosuppressing agents, such as methotrexate, act to turn off the T-cells. 

Chimeric antigen receptor (CAR) T-cell therapy is a treatment that uses a patient’s own T-cells which are modified with CARs. The CARs are created artificially and are engineered to recognize specific parts of the patient’s cancer cells. When the CARs are put into the patient’s T-cells, and the CAR-T cells transfused into the patient, these cells have the ability to recognize the cancer cells and kill them. This therapy approach would not work for LGL leukemia because T-cells are LGLs. There are generally few normal/healthy T-cells in LGL leukemia patients, and the leukemic ones are not normal. In addition, it would be difficult to target leukemic LGL using the few normal LGL in patients, since these cells are so similar. 

When is the immunosuppressant prednisone used in LGL leukemia treatment?

If a patient with LGL leukemia needs treatment, the immunosuppressants methotrexate, cyclophosphamide, or cyclosporine are typically used. These treatments suppress the immune system from making harmful molecules that destroy healthy cells. High-dose prednisone is also an immunosuppressant, but it works more quickly and thus can be better for short-term improvement of symptoms. It can be taken with methotrexate, then tapered off. However, long-term use of high-dose prednisone is not recommended due to the side effects.

In what situations is a bone marrow aspirate and biopsy performed to diagnose LGL leukemia?

A bone marrow aspirate and biopsy is not always necessary to make a diagnosis of LGL leukemia. The definition of LGL leukemia is an increased number of LGL cells in the blood (as determined by the peripheral blood smear and/or flow cytometry), along with confirming a clone is present (TCR test). Most patients with LGL leukemia can receive a definitive diagnosis with these tests. However, there are some situations in which a bone marrow aspirate and biopsy can be helpful. Below are several examples illustrating when a physician may want a bone marrow aspirate and biopsy performed:

  1. If diagnoses other than LGL leukemia are being considered (for example: myelodysplastic syndrome, aplastic anemia, pure red cell aplasia).
  2. If the number of LGL cells in the blood is not increased but other features are consistent with the diagnosis of LGL leukemia, then a bone marrow aspirate and biopsy can show that there is a clonal LGL cell population in the bone marrow.
  3. If the patient has had previous treatments that have not been very successful, a bone marrow aspirate and biopsy can confirm diagnosis of LGL leukemia and make sure there has not been an evolution to another disease (for example: myelodysplastic syndrome, aplastic anemia, pure red cell aplasia). 

My neutrophil count is variable (both on and off treatment) and that makes me nervous. Why do the numbers fluctuate?

The major goal of treatment in LGL leukemia is to increase the absolute neutrophil count (ANC) to a level where the patient is protected from life-threatening infections.  That level is an ANC greater than 500 (>500).  So, if a patient on treatment has an ANC that is persistently >500, then the medicine is working.  It is natural to have fluctuations in ANC; this happens in  individuals without LGL leukemia, too.  In addition, LGL leukemia patients take a very low dose of methotrexate, so it works slowly.  Therefore, it takes a long period of time for ANC to increase.  If an LGL leukemia patient is tolerating the medicine well, we continue methotrexate for at least 4 months.  At the 4 month interval, we expect the ANC to be >500 (this would be the case if methotrexate is working).  We are not concerned that much about the ANC levels before the 4 month period is up.  If we determine the methotrexate is working well after 4 months, then we recommend taking it indefinitely.  In some patients, we see even better responses, with normal blood cell counts being achieved at 1 to 2 years of treatment. 

The emphasis here is that the major benefit of methotrexate is keeping ANC >500.  A good response does not require all of the blood cell counts to become totally normal.

Is there a particular diet I should follow after my LGL leukemia diagnosis?

It is safest for you to speak with your healthcare provider so that you can learn what the best diet may be for your situation. 

  • Generally, eating a well-balanced diet is a good way to make sure you are getting appropriate nutrition. 
  • If possible, limit eating processed foods that may not provide as much nutrition as a whole food. For example, prepare your own meals or snacks from whole grains and fresh fruits, vegetables, and meats. 
  • If you decide to eat organic food, look for the “USDA Organic” label. This means the product met a strict organic standard set by the United States Department of Agriculture. 
  • If your healthcare provider has diagnosed you with celiac disease or said you have gluten sensitivity, it is best to avoid foods with gluten. If you do not have these conditions, eliminating gluten in your diet may or may not improve your health. In this case, the overall nutritional quality of a food may be more important to consider than whether or not it contains gluten.
  • If you are lactose intolerant or have been told by your doctor to eliminate/reduce dairy consumption, then it is best to avoid dairy products.  Otherwise, eliminating dairy from your diet may or may not improve your health.   
  • If your doctor has said you can consume alcohol, do so in moderation and responsibly. 

Will taking vitamin supplements help with my LGL leukemia symptoms?

It is always best to speak with your healthcare provider about your particular supplement needs. 

  • If your provider has checked the vitamin levels in your blood, and you have documented deficiencies (such as B or D vitamin deficiencies), you may wish to ask your healthcare provider about taking a supplement. Your provider can tell you how much to take to get your levels up to the normal range. You will probably have follow-up blood tests to make sure the supplement dose is appropriate. 
  • A daily multivitamin can provide a range of vitamins and minerals. There are many different types of multivitamins, so you may wish to ask your healthcare provider about which one is best for you. 
  • It is important to keep in mind that supplements are not reviewed by the Food and Drug Administration (FDA), so the health benefits on the supplement bottles are not verified. 

Is there a particular exercise that is most beneficial for people with LGL leukemia?

You should talk to your healthcare provider before starting any type of exercise program.

  • Importantly, while there are many types of exercises available, everyone is different and you may need to try a few things before finding an activity that you feel improves your overall health. 
  • Moderate walking for 30 minutes several times a week can help you relieve stress while providing you with a workout. 
  • Swimming is another good option for getting exercise.  This also may be beneficial if you have arthritis, because there is little impact on your joints while your muscles get a good workout. 
  • Some people find yoga is stress-relieving and builds strength. You can see if your local gym has a beginner’s yoga class to help you get started.
  • More strenuous types of exercises include running, weight-lifting or other sports. If you have never done these, you may want to consult with a trainer after approval from your healthcare provider. 

Is it safe for an LGL leukemia patient to get the Shingrix vaccination?

Yes, it is safe to get the Shingrix vaccination if you have LGL leukemia because it not a live virus vaccine. Shingrix iwas recently approved by the FDA for the prevention of shingles (herpes zoster) in people who are 50 years or older and who have previously had chicken pox.