Diffuse Large B Cell Lymphoma: Symptoms, Causes, Treatment

What is diffuse large B-cell lymphoma (DLBCL)? One of the most frequently asked questions by many people around the world. DLBCL is a rapidly growing blood cancer. It occurs when certain white blood cells, called B cells, multiply uncontrollably without properly maturing. 

DLBCL can affect people at any age, but it's more common in the mid-60s and slightly more common in men. This article provides important information about what causes DLBCL, the signs to look out for, factors that increase the risk, how it is diagnosed, and the available treatment options.

Diffuse large B cell lymphoma is a fast-growing cancer that affects B cells, a type of white blood cell responsible for producing antibodies.
It is a subtype of non-Hodgkin lymphoma (NHL), a cancer that affects the lymphatic system, which is a crucial part of our immune system.

Notably, the lymphatic system has vessels that carry a fluid called lymph, which contains special white blood cells called lymphocytes.
There are two main types of lymphocytes, B cells and T cells. 

In DLBCL, the abnormal B cells start to accumulate in lymph nodes or other organs in the body. This can cause swelling of the lymph nodes and the formation of lumps under the skin.
If left untreated, DLBCL can spread to other parts of the body, such as bone marrow, liver, or spleen.

Diffuse large B cell lymphoma is the most common type of NHL, accounting for 25% to 30% of all cases.
In adults, DLBCL is most commonly diagnosed in people over the age of 65. However, it can occur at any age. 

In children, DLBCL is known as paediatric diffuse large B cell lymphoma. It is commonly diagnosed in children under the age of 10.
However, it can occur at any age. The risk of developing DLBCL in children is not well understood.

In DLBCL, alterations or changes in genes and chromosomes can lead to different types or subtypes of the disease.
This causes DLBCL to vary from person to person. And these specific genetic changes influence how cancer behaves and the type of lymphoma it becomes. 

DLBCL - NOS (Not Otherwise Specified) is the most common category of DLBCL. It is given this name because it is a diagnosis used when DLBCL cases don't match any specific subgroups.

DLBCL - NOS are of two forms:

1. Germinal centre B cell-like (GCB) DLBCL: Diffuse large B cell lymphoma GCB type is the most common subtype, accounting for about 70% of cases.
   It is thought to arise from B cells that have undergone a normal process of development in the germinal centre of a lymph node. 

   GCB DLBCL is typically associated with a better prognosis than activated B cell-like DLBCL.

2. Activated B cell-like (ABC) DLBCL: Diffuse large B cell lymphoma activated B cell type is the less common subtype, accounting for about 30% of cases. It is thought to arise from B cells that have been activated by an infection or other stimuli. 

   ABC DLBCL is typically associated with a worse prognosis than GCB DLBCL.

There are also rare subtypes of diffuse large B cell lymphoma. While they may cause different symptoms compared to the common subtypes of DLBCL, they can often be treated in a similar manner.

Rare types of DLBCL: 

1. T Cell/Histiocyte-rich Large B Cell Lymphoma (TCRLBCL): This subtype of DLBCL involves the presence of T cells, histiocytes, and large B cells.
   It commonly affects middle-aged individuals and can resemble Hodgkin’s lymphoma.

2. Epstein-Barr Virus-positive DLBCL of the Elderly: This subtype of DLBCL typically occurs in individuals over the age of 50 and is associated with the Epstein-Barr virus (EBV).
   It can develop outside the lymph nodes or within them.

3. ALK-positive Large B Cell Lymphoma: This rare subtype involves a gene rearrangement called anaplastic lymphoma kinase (ALK).
   It often appears as localised lymphoma in lymph nodes or other organs. 

4. Primary Mediastinal (thymic) Large B Cell Lymphoma (PMBL): This type of DLBCL arises in the mediastinum, the area of the chest that contains the heart, lungs, and other organs.
   PMBL is more common in young women than in other groups.

5. Intravascular Large B Cell Lymphoma (IVLBCL): This type of DLBCL grows inside blood vessels.
   IVLBCL is often treated with chemotherapy and radiation therapy.

6. Diffuse Large B Cell Lymphoma CNS: It refers to a specific subtype of DLBCL that primarily affects the central nervous system (CNS), which includes the brain and spinal cord.
   It is a rare form of lymphoma. 

Diffuse Large B Cell Lymphoma Stages

Diffuse large B cell lymphoma has four stages to assess the extent of disease progression.
The stage of DLBCL is determined by factors like the size and quantity of affected lymph nodes, whether cancer cells are found in the bone marrow, and if cancer cells are present in other organs. 

Here are the four stages of DLBCL: 

1. Stage I: The cancer is in one lymph node region or one organ outside of lymph nodes.

2. Stage II: The cancer has spread to the same side of the diaphragm (the muscle separating the chest and abdomen) or one organ and nearby lymph nodes.

3. Stage III: The cancer has spread to lymph node regions on both sides of the diaphragm or extends from lymph nodes to nearby organs.

4. Stage IV: The cancer has spread extensively to multiple organs throughout the body, such as bone marrow, liver, lungs, or other distant sites.

The most common symptom that a patient with diffuse large B cell lymphoma will usually notice is swollen lymph nodes in their neck, armpits, abdomen, or testicles (in men).
These swellings may feel like a lump or mass that doesn't go away and appears to be growing larger. 

Apart from enlarged lymph nodes, the other major symptoms of DLBCL include:     

1. Unexplained Fever: Frequent episodes of fever that come and go for no apparent reason.

2. Heavy Night Sweats: Experiencing excessive sweating during the night that is intense and profuse.

3. Unexplained Weight Loss: Significant reduction in body weight (losing more than one-tenth of the total weight) without a known cause or intentional effort.

The symptoms experienced due to DLBCL can vary depending on its location in the body. These are called local symptoms. 

DLBCL in the stomach or bowel can cause:

1. Abdominal discomfort or pain

2. Nausea

3. Diarrhoea

4. Bleeding

DLBCL in the chest can cause:

1. Breathlessness

2. Cough

3. Chest pain

4. Headache

DLBCL is a type of non-Hodgkin lymphoma that can develop due to various factors. While the exact cause of DLBCL is unknown, several risk factors have been identified that may contribute to its development. 

Diffuse Large B Cell Lymphoma Risk Factors

Below mentioned are some of the risk factors that may increase the likelihood of developing DLBCL: 

1. Age: The risk of developing DLBCL increases with age. The average age at diagnosis is 65 years old, but it can occur at any age.

2. Gender: DLBCL is more common in men than in women.

3. Family History: People with a family history of DLBCL are at an increased risk of developing the disease.

4. Certain Infections: Some infections involving the Epstein-Barr virus, hepatitis B virus, and hepatitis C virus have been linked to an increased risk of DLBCL development.

5. Certain Autoimmune Disorders or Immunodeficiency: Taking immunosuppressant drugs after organ transplants to prevent rejection, and having autoimmune disorders like HIV/AIDS, rheumatoid arthritis or Sjögren's syndrome, can weaken the immune system and make it less capable of fighting infections, potentially increasing the risk of DLBCL.

6. Certain Environmental Factors: Ionising radiation is a type of radiation that can damage DNA. Agricultural chemicals or pesticides can also damage DNA.  

7. Previous Treatments: Before radiation and chemotherapy treatment, exposure to higher radiation levels can increase the chances of developing DLBCL.

There are currently no specific guidelines for preventing diffuse large B cell lymphoma, as the exact causes of this type of lymphoma are not fully understood.
However, one can take the following preventive measures to potentially reduce the risk of developing DLBCL:   

1. Avoid exposure to chemicals like herbicides, pesticides, and benzene.

2. If an individual has celiac disease, which is an autoimmune disorder triggered by gluten, then he/she should maintain a gluten-free diet.
   This diet will help minimise the stimulation of their immune system, potentially reducing the risk of lymphomas like DLBCL.

3. Follow a healthy lifestyle with regular exercise and a balanced diet.

4. Seeking medical attention promptly if any unusual symptoms or persistent changes are observed can lead to early detection and timely treatment.

Diagnosing DLBCL involves a comprehensive evaluation that includes several diagnostic tests and procedures.
The aim is to confirm the presence of DLBCL, determine the extent of the disease, and guide appropriate treatment decisions.
Here are some commonly used diagnostic approaches for DLBCL:

1. Patient History: A healthcare provider will inquire about the patient's medical history, including any previous cancer treatments, as well as their symptoms, such as fever, night sweats, weight loss, and fatigue.

2. Clinical Examination: A physical examination will be conducted by the doctor, checking the patient's lymph nodes, liver, and spleen. 

Common tests for diagnosis of DLBCL include: 

1. Biopsy: It is a diagnostic procedure in which a swollen lymph node is removed, or a tissue sample is taken for examination.
   The obtained tissue sample is then analysed under a microscope to confirm the DLBCL diagnosis and guide the selection of appropriate treatment.

2. Imaging Tests: Imaging tests, such as PET, CT and MRI scans, are essential for identifying the affected areas and providing information about the size and location of the lymphoma. 

3. Blood Tests: These tests provide a comprehensive assessment of general health.
   Tests such as complete blood count (CBC) evaluate blood cell counts, while blood chemistry tests assess kidney and liver function and screen for infections like HIV, hepatitis B, and hepatitis C. 

4. Bone Marrow Biopsy: A bone marrow biopsy is done to check if there are lymphoma cells in the bone marrow. This involves collecting a sample of bone marrow cells for examination.

5. Lumbar Puncture: Sometimes, a lumbar puncture is performed to examine the fluid around the brain and spinal cord (cerebrospinal fluid or CSF).
   It helps determine if there are lymphoma cells present in CSF.

How to Prepare for the Doctor Consultation?

A doctor consultation is a valuable opportunity to discuss health concerns, seek medical advice, and develop a collaborative action plan with the healthcare provider.

Preparing for this appointment can maximise one's time and ensure a productive and informative discussion. Here are some basic things to prepare: 

1. Individuals should schedule a prior appointment with the doctor,

2. Make a list of medications and supplements that the patient consumes.

3. Make a note of all the symptoms experienced by the patient.

4. Bring relevant medical records during the consultation with the doctor. 

5. Also, make a note of all the relevant doubts and questions. Here are some of the questions that a patient can ask the doctor if diagnosed with DLBCL:

1. What is the stage of the DLBCL?

2. What is the prognosis for DLBCL?

3. What can they do to manage their symptoms?

4. What are the treatment options available for DLBCL?

5. Are there any side effects of the treatment?

6. How can they manage the side effects of treatment?

Due to the aggressive nature of diffuse large B cell lymphoma, treatment is usually initiated promptly after diagnosis.
The specific approach for the treatment of DLBCL may vary based on factors such as the stage of DLBCL, the patient's overall health, and any other risk factors.
A team of doctors, including oncologists, haematologists, and radiologists, work together to make treatment decisions.  

1. Chemotherapy and Immunotherapy: 

1. DLBCL is treated with chemoimmunotherapy, combining chemotherapy and immunotherapy drugs. 

2. The standard treatment option for diffuse large B cell lymphoma is R-CHOP, which involves a combination of rituximab (a monoclonal antibody), three chemotherapy drugs, and a drug that aids in eliminating lymphoma cells. 

3. Treatment is given in cycles over a few weeks. The number of cycles depends on the stage and risk.
   Sometimes, longer or more intensive treatment may be recommended. The plan may be adjusted based on age and health.

4. This treatment is very effective for treating DLBCL, but it can cause a number of side effects, and there is also a risk of relapses.  

2. Radiotherapy: Radiation therapy uses high-energy rays to kill cancer cells. Radiation therapy is given in fractions, each lasting 15-30 minutes.
   The number of fractions depends on the stage of diffuse large B cell lymphoma and treatment response.
   This treatment may be recommended either after chemotherapy to prevent the lymphoma from recurring or prior to a stem cell or bone marrow transplant.

3. Surgery: It is not commonly used for DLBCL treatment. However, it may be done to remove large tumours or relieve symptoms caused by a mass pressing on an organ and causing a blockage.
   It may be used in combination with other treatments.

4. Stem Cell Transplantation: Stem cell or bone marrow transplants involve using stem cells from either the individual themselves or a donor to allow for high-dose chemotherapy treatment.
   Transplants are considered for diffuse large B cell lymphoma in cases of potential relapse, second remission, or unresponsiveness to other treatments.

Cost of Diffuse Large B Cell Lymphoma Treatment

The cost of diffuse large B cell lymphoma treatment in India varies based on several factors, including the type and stage of cancer, the patient’s age, the patient's comorbidities, the type of treatment modality, type and location of the hospital, doctor’s expertise, insurance coverage and much more.

To know the exact cost of DLBCL treatment in India, one needs to consult their healthcare provider.
However, the below table also provides the approximate cost of various treatment techniques used for the DLBCL treatment.

Diffuse Large B Cell Lymphoma Survival Rate

The survival rate for patients with diffuse large B cell lymphoma varies depending on a number of factors, including the patient's age, stage of disease, and IPI score, which is a risk prediction tool that is used to assess a patient's overall prognosis.

The table provided by the National Institute of Health (NIH) in the United States presents the 10-year overall survival (OS) rates for patients with DLBCL, stratified by IPI risk group. 

In other words, patients with a low IPI score are more likely to survive for ten years after diagnosis than patients with a high IPI score.
Please note that the IPI score is a risk prediction tool, not a guarantee of survival. 

The American Cancer Society provides 5-year survival rates for DLBCL patients based on their stage, using data from the Surveillance, Epidemiology, and End Results (SEER) database. 

Notably, the SEER staging system is a way to categorise how far cancer has spread and is used for many types of cancer, including diffuse large B cell lymphoma.

SEER staging for DLBCL consists of three main categories: 

1. Localised: Cancer is only in one area and hasn't spread to nearby lymph nodes or other parts of the body.

2. Regional: Cancer has spread to nearby lymph nodes or tissues but hasn't reached distant areas.

3. Distant: Cancer has spread to organs or sites far away from where it started, indicating a more advanced stage.

SEER staging helps doctors understand the extent of cancer and decide on the best treatment options.
It's also useful for tracking and studying cancer patterns and outcomes.

It is important to remember that survival rates are estimates based on the experiences of large groups of people with different forms of DLBCL at different stages of their lives and with varying overall health.
Healthcare providers serve as the best resource for DLBCL patients seeking information about their specific prognosis and what they may expect.

Diffuse large B cell lymphoma can give rise to various complications that can impact different organs and systems in the body.
These complications can come from cancer cells themselves or the treatments used to fight the disease. 

Some common complications include: 

1. Tumour Lysis Syndrome (TLS): TLS is a life-threatening complication that can occur in patients with DLBCL and other types of cancer.
   It is caused by the rapid breakdown of tumour cells, which releases harmful substances from dying tumour cells into the bloodstream.
   This causes various serious complications, including kidney failure, heart arrhythmias, and seizures.

2. Gastrointestinal Problems: Symptoms like nausea, vomiting, diarrhoea, and constipation may occur when DLBCL affects the gastrointestinal tract.

3. Renal Complications: Although uncommon, DLBCL can lead to renal failure when it involves kidneys.

4. Neurological Issues: Headache, seizures, weakness, and vision problems may arise if DLBCL spreads to the central nervous system.

5. Bone Marrow Complications: DLBCL spreading to the bone marrow can cause bone marrow problems, such as anaemia, thrombocytopenia, and neutropenia.

1. Anaemia: The disease can cause a lack of red blood cells, leading to fatigue, shortness of breath, and pale skin.

2. Thrombocytopenia: It can cause a lack of platelets, leading to bleeding, bruising, and petechiae.

3. Neutropenia: It is a condition in which the body does not have enough neutrophils, which are a type of white blood cells that helps the body fight infection. 

Risks if Not Treated in Time 

If left untreated, diffuse large B cell lymphoma poses significant risks. Timely intervention is vital for better outcomes.
Risks of untreated DLBCL include:

1. Spread of Cancer: DLBCL can spread to other parts of the body, making it harder to treat and control the disease.

2. Organ Damage: As DLBCL progresses, vital organs like the liver, kidneys, lungs, or central nervous system can be affected, causing serious complications and impairing normal function.

3. Worsening Symptoms: Untreated DLBCL can result in symptoms like fatigue, weight loss, night sweats, and persistent fever, which can worsen over time and impact a person's quality of life.

4. Reduced Survival Rate: DLBCL is an aggressive lymphoma, and without timely treatment, the chances of achieving remission and long-term survival decrease significantly.

5. Death: If left untreated, DLBCL can be fatal, and the risk of death increases the longer it remains untreated.

When to See a Doctor?

Early diagnosis and treatment of diffuse large B cell lymphoma can improve the chances of a good outcome. It is important to be observant of the changes in your body.
Below mentioned are the signs and symptoms that indicate you must visit a doctor soon: 

1. Persistent and unexplained symptoms such as unexplained weight loss, fatigue, or night sweats

2. Enlarged lymph nodes that do not go away or continue to grow in size

3. Unexplained pain or discomfort, especially in the abdomen, chest, or bones

4. Neurological symptoms such as headache, seizures, or weakness

5. Persistent gastrointestinal symptoms such as abdominal pain, vomiting, or changes in bowel habits

6. Any concerning changes in vision or eye-related symptoms

7. Signs of infection, such as persistent fever, cough, or difficulty breathing

If someone is experiencing any of these symptoms, it is important for them to consult a doctor for proper evaluation and diagnosis, as other conditions can also cause these symptoms.

While there isn't a specific diet for diffuse large B cell lymphoma, following these general guidelines can be helpful for individuals with the condition.

Patients should consume a variety of fruits and vegetables daily.

1. They should choose whole grains for their carbohydrate sources.

2. They should include protein from diverse sources such as meat, fish, eggs, dairy items, beans, nuts, and seeds.

3. They should increase their fibre intake through plant-based foods like fruits, vegetables, cereals, and potatoes.

4. They should opt for healthy fats found in seeds, nuts, olives and avocados.

5. They should stay adequately hydrated by drinking enough fluids throughout the day, excluding alcohol.

Individuals should consult with a healthcare professional or dietitian who can provide personalised guidance and a diet plan based on their specific circumstances, dietary restrictions, treatment, and other conditions.

In conclusion, Diffuse Large B Cell Lymphoma (DLBCL) is a complex and aggressive form of non-Hodgkin lymphoma. Prompt diagnosis and appropriate treatment are crucial for achieving favourable outcomes.
Diagnostic procedures such as medical history, physical examination, imaging tests, biopsies, immunophenotyping, and molecular/genetic testing play a vital role in confirming the presence of DLBCL, determining its subtype, and assessing the extent of the disease.

If an individual is experiencing symptoms of DLBCL, prompt medical attention should be sought. HexaHealth, with its extensive network of hospitals offering a range of surgeries, can provide assistance in finding the right doctors and hospitals for specific needs.
Through HexaHealth's advanced AI-powered recommendation engine, individuals can be connected with skilled surgeons who specialise in DLBCL treatment, ensuring they receive the finest care available.

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