A rare type of chronic (long-term) leukemia that negatively affects blood cells called lymphocytes is called large granular lymphocytic leukemia (LGL). In general, there are 3 types of large granular lymphocytic leukemia. While two of them grow slowly and are very similar to certain chronic disorders, the third type is aggressive. It means it grows quickly and may spread to other parts of the body. Unfortunately, there is no way to cure this type of cancer. That’s why the treatment goal is to ease the symptoms and prevent life-threatening complications.
In other words, LGL is a type of leukemia that affects white blood cells (lymphocytes) that normally fight viruses, bacteria, and other germs that can make you ill. Types of LGL that grow slowly include T-cell large granular lymphocytic leukemia (T-LGL) and chronic lymphoproliferative disorder of NK cells (CLPD-NK). The tumors often develop in white blood cells known as cytotoxic T cells or natural killer cells.
In general, LGL occurs very rarely, and it affects about 1 in 1 million people worldwide.
Symptoms
Some people with LGL do not have symptoms. According to one study, approximately 33% of people with LGL did not have any symptoms when they were diagnosed. In rare cases, people who were treated for blood disorders are diagnosed with LGL. In this case, people experience symptoms that are often linked with LGL. Check some symptoms below:
- Fatigue (extreme tiredness) – This is one of the most common symptoms of LGL that happens due to anemia (low red blood cell count).
- Frequent infections and fevers – They are often caused by bacterial infections.
- Enlarged spleen (also known as splenomegaly) – The spleen can become enlarged due to certain forms of anemia and infections.
If any of the previous symptoms occur, immediately contact your healthcare provider. Otherwise, it may lead to life-threatening complications.
What Health Conditions Are Often Linked to Large Granular Lymphocytic Leukemia?
A lot of people who develop LGL also have autoimmune disorders. Check some examples below:
- Rheumatoid arthritis – This is a long-term (chronic), autoimmune disorder in which the immune system mistakenly attacks the joint lining (also known as synovium), causing painful inflammation.
- Anemia – This health condition occurs when a person does not have enough red blood cells and hemoglobin. Normally, red blood cells carry oxygen to all tissues and organs in the body. However, most people with LGL have hemolytic anemia. It happens because red blood cells are destroyed by the cancer and not because of reduced production.
- Lymphocytosis – This condition occurs when there are high levels of lymphocytes in the blood. Those who suffer from lymphocytic leukemias, lymphomas, and viral infections usually have reduced lymphocyte counts.
Causes and Risk Factors
Nowadays, experts continue to study possible causes of LGL. However, they believe there is a connection between this form of leukemia and autoimmune disorders or other types of cancer. Examples include:
- About 30% of people with LGL also have rheumatoid arthritis or other autoimmune disorders.
- Roughly 25% to 30% also have another type of lymphoma.
- Most people who develop LGL also carry two mutated genes, including STAT3 and STAT5B. Normally, these genes are responsible for dividing and multiplying the immune cells.
While anyone can develop this disorder, there are some factors that could increase your risk of developing it. Check some risk factors below:
- Autoimmune disorders – This is one of the most significant risk factors for LGL. The most common condition found in people with this type of leukemia is rheumatoid arthritis, but other people may develop Sjogren’s syndrome and systemic lupus erythematosus.
- Age – Usually, LGL affects older adults (over 55 years old).
- Genetic factors – A mutation in one of the following genes may also contribute to LGL. For example, STAT3 and STAT5b.
- Chronic (long-term) inflammation – People who have chronic inflammatory disorders for long periods or who leave them untreated are also at increased risk for T-cell LGL.
What Are The Long-term Effects of LGL?
People who develop LGL may also experience some complications, especially if the condition is found in advanced stages or is left untreated. Check some examples below:
- Neutropenia and infections – While neutropenia is one of the most common and serious complications of LGL, some people may also experience severe infections (such as pneumonia or sepsis).
- Anemia – This condition negatively affects the amount of red blood cells in the body. These cells are responsible for carrying oxygen to all tissues and organs in the body.
- Autoimmune disorders – While these conditions can contribute to LGL, this type of cancer may also increase your risk for certain autoimmune disorders (such as lupus).
- Splenomegaly and hepatomegaly – Approximately 20% of patients experience low platelets (thrombocytopenia), which may lead to easy bruising.
- Systemic symptoms (including severe night sweats, unusual weight loss, fever, and others)
- Pulmonary hypertension
- Neuropathy
- Hemophagocytic syndrome
This document does not contain a complete list of LGL complications. In any case, you can talk with your doctor about measures to reduce the risk or even prevent them. Moreover, there is no way to prevent this type of cancer.
Diagnosis
Most of the time, physicians begin the diagnosis with blood and genetic tests. They may also perform the following tests to get more clues about your disease. Examples include:
- Peripheral blood smear – This test is used to examine blood cells under a microscope.
- Complete blood count (CBC) with differential – This type of blood test is used to measure all of the blood cells (including the amount of each type of white blood cell).
- Flow cytometry – This is a lab test that helps doctors analyze cell characteristics. Commonly, doctors perform it to classify types of leukemia.
- Immunophenotyping – This test helps check for markers on cell surfaces.
- T-cell receptor (TCR) gene rearrangement analysis
- Genetic testing – During this test, physicians will take a sample of blood to check for mutations in the STAT3 and STAT5 genes.
Sometimes, physicians may perform additional tests to exclude the following health conditions. For example, immunodeficiency, rheumatoid arthritis, myelodysplasia, and myeloid mutations.
Treatment
Doctors often do not recommend any treatments if you have this type of cancer and do not have symptoms. Thus, they will monitor this condition for a few months for signs and symptoms. However, if you have symptoms, doctors often prescribe immunosuppressive therapy and steroids that often help relieve the symptoms and prevent life-threatening complications.
Frequently Asked Questions
Is large granular lymphocytic leukemia a fatal illness?
Mostly, LGL is a long-term condition that is not fatal. Approximately 75% of people with T-LGL leukemia and CLPD-LGL leukemia are alive 5 years after diagnosis. However, about 10% of people who are diagnosed with these forms of leukemia die of complications (such as severe infections).
Can you live a normal life with this condition?
Commonly, people who get treatment live normal lives and have the same lifespan as people without LGL. Unfortunately, some people with LGL develop serious blood disorders that negatively affect their life expectancy. For more details, discuss it with your doctor.
How do I take care of myself?
Physicians may recommend some tips to manage the symptoms and improve your quality of life. These include:
- Regularly perform physical exercise
- Get plenty of rest and fluids
- Manage stress and chronic health conditions (such as diabetes, high blood pressure, high cholesterol, and others)
- Adopt a healthy diet that includes lean protein, fruits, vegetables, and whole grains
Can large granular lymphocytic leukemia go into remission?
Yes. Some people with LGL achieve long periods of remission after treatment, especially when symptoms and abnormal blood counts improve significantly. However, because LGL is usually a chronic condition, ongoing monitoring is often necessary even when the disease is well controlled.
Is LGL considered an inherited disease?
No, LGL is not generally considered an inherited cancer. Most genetic mutations associated with LGL, such as changes in the STAT3 or STAT5B genes, develop during a person’s lifetime and are not typically passed from parents to children.
How often should people with LGL see their doctor?
The frequency of follow-up visits depends on the severity of the disease and the treatment plan. People without symptoms may only need periodic blood tests and examinations, while those receiving treatment often require more frequent monitoring to evaluate blood cell counts and treatment effectiveness.
Can LGL transform into a more aggressive cancer?
In most cases, the chronic forms of LGL remain stable and progress slowly. However, in rare cases, it may become more aggressive or cause severe complications. Regular medical follow-up helps healthcare providers detect any concerning changes early.
What foods should people with LGL eat?
Although no specific diet can cure LGL, a balanced diet rich in fruits, vegetables, whole grains, lean proteins, and healthy fats can support overall health and immune function. People with low white blood cell counts should also follow their doctor’s recommendations regarding food safety to reduce the risk of infections.
Can LGL affect platelet counts?
Some people with LGL develop thrombocytopenia, a condition characterized by low platelet levels. Platelets help blood clot properly, and reduced levels may increase the risk of bruising, prolonged bleeding, or frequent nosebleeds.
When should I contact a healthcare provider if I have LGL?
You should contact your physician if you experience persistent fever, signs of infection, unusual bleeding, severe fatigue, shortness of breath, unexplained weight loss, or worsening of other symptoms. Prompt medical attention can help prevent serious complications and improve treatment outcomes. Ask your healthcare provider if you have additional questions.
