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Diffuse Large B Cell Lymphoma (DLBCL): symptoms, Causes, Stages and Treatment  | Max Hospital

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Diffuse Large B-cell Lymphoma: Causes, Diagnosis, and Treatment

By Dr. Amrita Chakrabarti in Hematology Oncology

Jun 14 , 2024 | 13 min read

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In recent years, cancer research and treatment advancements have significantly improved outcomes for patients facing various forms of cancer. Among these is Diffuse Large B-cell Lymphoma (DLBCL), a prevalent and aggressive type of non-Hodgkin lymphoma. DLBCL presents unique challenges in both its diagnosis and treatment, prompting an ongoing quest for deeper understanding and more effective therapeutic approaches. In this article, we delve into the causes, diagnosis, and treatment modalities associated with DLBCL, exploring the intricate interplay between medical science, patient care, and the pursuit of better outcomes. Let’s begin with some basics.

What is Diffuse Large B-Cell Lymphoma?

Diffuse large B-cell lymphoma is a type of cancer that originates in the lymphatic system, which is part of the body's immune system. It is the most common type of non-Hodgkin lymphoma, accounting for about one-third of all cases. Characterized by the rapid growth of large B-cell lymphocytes, a type of white blood cell, in lymph nodes and other lymphoid tissues, DLBCL can occur at any age but is more commonly diagnosed in older adults. 

How does Diffuse Large B-Cell Lymphoma Affect the Body?

Diffuse large B-cell lymphoma (DLBCL) affects the body primarily through its impact on the lymphatic system and immune function. Here's how it typically affects the body:

  • Lymph node enlargement: DLBCL often manifests as painless swelling of lymph nodes, most commonly in the neck, armpits, or groin. This enlargement occurs due to the accumulation of cancerous B-cells within the lymph nodes.
  • Systemic symptoms: Patients with DLBCL may experience systemic symptoms such as fever, night sweats, fatigue, and unintentional weight loss. These symptoms can be indicative of the body's immune response to the presence of cancerous cells and the release of cytokines and other signaling molecules.
  • Compression symptoms: In some cases, enlarged lymph nodes in DLBCL can compress nearby structures, leading to symptoms such as difficulty breathing or swallowing, coughing, or abdominal pain if lymph nodes in the chest or abdomen are affected.
  • Infiltration of organs: DLBCL can infiltrate other organs besides lymph nodes, such as the spleen, liver, bone marrow, or central nervous system. This infiltration can disrupt normal organ function and lead to additional symptoms depending on the affected organ.
  • Immune dysfunction: Since DLBCL originates from B-cells, which are crucial components of the immune system, the disease can impair the body's ability to mount an effective immune response against infections and other diseases. This can make patients more susceptible to infections and may require careful management during cancer treatment.

Overall, DLBCL affects the body by disrupting normal lymphatic and immune function, leading to a range of symptoms that can vary depending on the extent of the disease and its impact on different organs and systems. 

What are the Stages of Diffuse Large B-cell Lymphoma?

Diffuse large B-cell lymphoma is staged based on the extent of the disease at the time of diagnosis. The staging system commonly used for DLBCL is the Ann Arbor staging system, which classifies lymphomas into four stages:

  1. Stage I: In Stage I DLBCL, the cancer is localized to a single lymph node region or a single extralymphatic site.
  2. Stage II: In Stage II DLBCL, the cancer involves two or more lymph node regions on the same side of the diaphragm or extends from one lymph node region into a nearby organ or tissue.
  3. Stage III: In Stage III DLBCL, the cancer involves lymph node regions on both sides of the diaphragm. It may also involve nearby organs or tissues.
  4. Stage IV: In Stage IV DLBCL, the cancer has spread extensively beyond the lymphatic system to distant organs or tissues, such as the bone marrow, liver, or lungs.

In addition to the stage, DLBCL is further classified based on other factors, including:

  • Bulky disease: DLBCL with bulky disease refers to the presence of large masses of cancerous tissue that measure more than 10 centimeters in diameter.
  • Extranodal involvement: DLBCL may involve extranodal sites, such as the gastrointestinal tract, central nervous system, bone marrow, or other organs.
  • International Prognostic Index (IPI): The IPI is a prognostic tool used to assess the risk of disease progression and overall survival in patients with DLBCL. It takes into account factors such as age, stage of disease, performance status, serum lactate dehydrogenase (LDH) level, and the number of extranodal sites involved.

What are the Symptoms of Diffuse Large B-cell Lymphoma?

Diffuse Large B-cell Lymphoma (DLBCL) can present with a variety of symptoms, which may vary from person to person. Some common symptoms of DLBCL include:

  • Painless swelling of lymph nodes: Enlarged lymph nodes, typically in the neck, armpits, or groin, are a common early symptom of DLBCL.
  • Fever: Fever, often with no obvious cause, may occur as a result of the body's immune response to the cancer.
  • Night sweats: Profuse sweating during sleep, unrelated to room temperature or external factors, is another common symptom of DLBCL.
  • Fatigue: Persistent tiredness or weakness that is not relieved by rest can be a symptom of DLBCL.
  • Unexplained weight loss: Significant weight loss without a clear cause or change in diet or activity level may occur in individuals with DLBCL.
  • Loss of appetite: Decreased appetite or a feeling of early satiety (feeling full quickly) can be associated with DLBCL.
  • Abdominal pain or discomfort: Enlarged lymph nodes or involvement of organs in the abdomen may cause pain, discomfort, or swelling in the abdominal area.
  • Shortness of breath or cough: In cases where DLBCL affects lymph nodes in the chest or infiltrates the lungs, symptoms such as shortness of breath, coughing, or chest pain may occur.

It's important to note that these symptoms can also be caused by other conditions, and the presence of these symptoms does not necessarily indicate DLBCL. However, if any of these symptoms persist or worsen, it's essential to consult a healthcare professional for a thorough evaluation and appropriate management.

What are the Causes and Risk Factors of B-cell Lymphoma?

While the exact cause is not fully understood, there are several factors that may contribute to its development. These include:

  • Genetic factors: DLBCL may arise due to genetic mutations that affect the normal functioning of B-cells, which are a type of white blood cell involved in the immune response.
  • Immune system dysfunction: Alterations in the immune system, such as chronic immunosuppression for autoimmune diseases, may increase the risk of developing DLBCL.
  • Infections: Certain infections, such as the Epstein-Barr virus (EBV) or the human immunodeficiency virus (HIV), have been associated with an increased risk of DLBCL.
  • Environmental factors: Exposure to certain environmental toxins, such as pesticides, solvents, or radiation, may contribute to the development of DLBCL, although the evidence is limited.
  • Age and gender: DLBCL is more common in older adults, with the risk increasing with age. Additionally, men are slightly more likely to develop DLBCL than women.
  • Other medical conditions: Some medical conditions, such as autoimmune diseases, chronic inflammation, or previous cancer treatments (e.g., chemotherapy or radiation therapy), may increase the risk of DLBCL.

It's important to note that not everyone with these risk factors will develop the disease. Additionally, many cases of DLBCL occur in individuals with no identifiable risk factors, indicating that the disease's development is likely multifactorial.

How do Doctors Diagnose Diffuse Large B-Cell Lymphoma?

The diagnosis of diffuse large B-cell lymphoma typically involves a combination of medical history review, physical examination, and various diagnostic tests. Here's an overview of the diagnostic process:

  • Medical history and physical examination: The doctor will ask about symptoms, medical history, and any risk factors. They will also conduct a physical examination to check for signs such as enlarged lymph nodes, fever, weight loss, and other symptoms commonly associated with DLBCL.
  • Blood tests: Blood tests, including a complete blood count (CBC) and blood chemistry tests, may be conducted to assess overall health and detect any abnormalities, such as anemia, abnormal white blood cell counts, or changes in organ function.
  • Imaging tests: Imaging studies such as CT scans, PET scans, or MRI scans may be ordered to visualize the extent of lymphoma and identify any affected areas in the body, such as lymph nodes, organs, or bones.
  • Biopsy: A biopsy is the definitive diagnostic test for DLBCL. It involves the removal of a small tissue sample (biopsy) from an enlarged lymph node or other affected area. The biopsy sample is then examined under a microscope by a pathologist to confirm the presence of lymphoma cells and determine the specific subtype, such as DLBCL.
  • Bone marrow biopsy: In some cases, a bone marrow biopsy may be performed to assess whether lymphoma has spread to the bone marrow. This involves removing a small sample of bone marrow from the hip bone and examining it for the presence of lymphoma cells.
  • Additional tests: Depending on the individual case, additional tests may be conducted to further characterize the lymphoma and assess its stage, including immunohistochemistry, flow cytometry, cytogenetic analysis, and molecular testing.

Once a diagnosis of DLBCL is confirmed, further tests may be performed to determine the extent of the disease (staging) and guide treatment planning. These may include additional imaging studies, such as a bone scan or lumbar puncture, as well as tests to assess organ function and overall health.

How do Doctors Treat Diffuse Large B-Cell Lymphoma?

Doctors typically treat diffuse large B-cell lymphoma using a combination of therapies tailored to the individual patient's condition and specific characteristics of the lymphoma. The primary treatment modalities for DLBCL include:

Chemotherapy

Chemotherapy is the cornerstone of treatment for DLBCL. It involves the use of powerful anti-cancer drugs to kill cancer cells or stop their growth. The most common chemotherapy regimen for DLBCL is called R-CHOP, which combines several drugs: rituximab (R), cyclophosphamide (C), doxorubicin (H), vincristine (O), and prednisone (P). Other chemotherapy regimens may be used depending on the patient's overall health and response to treatment.

Immunotherapy

Rituximab, a monoclonal antibody that targets a protein called CD20 found on the surface of B-cells, is often combined with chemotherapy in the treatment of DLBCL. This combination therapy, known as R-CHOP, has been shown to improve outcomes for many patients with DLBCL by enhancing the effectiveness of chemotherapy and reducing the risk of relapse.

Targeted therapy

For certain patients with DLBCL who have specific genetic mutations or molecular abnormalities, targeted therapies may be used. These drugs are designed to specifically target and inhibit the activity of cancer cells while sparing normal cells, potentially leading to fewer side effects. Examples of targeted therapies used in DLBCL include drugs that target the B-cell receptor signaling pathway, such as ibrutinib and lenalidomide.

Radiation therapy

Radiation therapy may be used in combination with chemotherapy for localized DLBCL or as a standalone treatment for patients who are unable to tolerate chemotherapy. It involves the use of high-energy X-rays or other forms of radiation to kill cancer cells and shrink tumors.

Stem cell transplantation

In some cases, particularly for patients with relapsed or refractory DLBCL or high-risk disease, stem cell transplantation may be considered. This procedure involves replacing diseased bone marrow with healthy stem cells, which can help restore the body's ability to produce healthy blood cells and fight off infections.

The specific treatment approach for DLBCL depends on factors such as the stage and extent of the disease, the patient's overall health and preferences, and the presence of any genetic or molecular abnormalities. Treatment decisions are typically made in collaboration between the patient and a multidisciplinary team of healthcare providers, including oncologists, hematologists, radiation oncologists, and other specialists.

What are Survival Rates for Diffuse Large B-Cell Lymphoma?

Survival rates for diffuse large B-cell lymphoma vary depending on several factors, including the stage of the disease, the patient's age and overall health, and the specific characteristics of the lymphoma. Overall, DLBCL has a relatively good prognosis, with many patients achieving long-term remission or cure with appropriate treatment. Here are some general survival rates:

  • 5-year survival rate: The overall five-year survival rate for DLBCL is estimated to be around 60% to 70%. This means that, on average, about 60% to 70% of patients diagnosed with DLBCL are still alive 5 years after diagnosis.
  • Localized disease: Patients with early-stage DLBCL (Stage I or Stage II) have a higher chance of cure, with 5-year survival rates ranging from 70% to 90% or higher, especially if they receive appropriate treatment, such as combination chemotherapy and radiation therapy.
  • Advanced disease: Patients with advanced-stage DLBCL (Stage III or Stage IV) typically have lower survival rates compared to those with early-stage disease. However, with advances in treatment, including more effective chemotherapy regimens and immunotherapy, survival rates for advanced-stage DLBCL have improved in recent years.
  • Prognostic factors: Several prognostic factors may influence survival outcomes in DLBCL, including the patient's age, performance status, LDH levels, and the presence of certain genetic or molecular abnormalities. Patients with favorable prognostic factors tend to have better survival outcomes than those with unfavorable prognostic factors.
  • Relapsed or refractory disease: For patients who experience a relapse of DLBCL or do not respond to initial treatment (refractory disease), survival rates may be lower. However, additional treatment options, such as salvage chemotherapy, targeted therapies, stem cell transplantation, or participation in clinical trials, may offer the possibility of long-term remission or cure for some patients.

It's important to note that survival rates are statistical estimates based on large populations of patients and may not accurately predict an individual patient's outcome. Each patient's situation is unique, and factors such as response to treatment, disease biology, and overall health can significantly influence their prognosis. Therefore, it's essential for patients to discuss their specific diagnosis and treatment options with their healthcare team to better understand their individual prognosis and make informed decisions about their care.

How to Reduce the Risk of Developing Diffuse Large B-Cell Lymphoma?

Reducing the risk of developing diffuse large B-cell lymphoma may involve adopting certain lifestyle habits and avoiding potential risk factors. While it's not always possible to prevent DLBCL, following these general guidelines may help reduce the risk:

  • Maintain a healthy lifestyle: Eating a balanced diet rich in fruits, vegetables, whole grains, and lean proteins, and maintaining a healthy weight can help support overall health and reduce the risk of various cancers, including lymphomas such as DLBCL.
  • Exercise regularly: Engaging in regular physical activity can help strengthen the immune system, maintain a healthy weight, and reduce the risk of certain cancers. Aim for at least 150 minutes of moderate-intensity exercise or 75 minutes of vigorous-intensity exercise per week.
  • Limit alcohol consumption: Excessive alcohol consumption has been associated with an increased risk of developing certain cancers, including lymphomas. Limit alcohol intake to no more than one drink per day for women and two drinks per day for men, as recommended by health authorities.
  • Avoid tobacco products: Smoking and tobacco use have been linked to various cancers, including lymphomas. Avoiding tobacco products and exposure to secondhand smoke can help reduce the risk of DLBCL and other smoking-related cancers.
  • Protect against infections: Some infections, such as the Epstein-Barr virus (EBV) and the human immunodeficiency virus (HIV), have been associated with an increased risk of DLBCL. Practicing good hygiene, getting vaccinated against infectious diseases when appropriate, and practicing safe sex can help reduce the risk of infection-related cancers.
  • Limit exposure to environmental toxins: Certain environmental toxins, such as pesticides, solvents, and industrial chemicals, have been linked to an increased risk of cancer, including lymphomas. Minimize exposure to environmental toxins whenever possible, and follow safety guidelines when working with hazardous materials.
  • Regular health checkups: Regular medical checkups and screenings can help detect any potential health issues early on, allowing for prompt diagnosis and treatment. Be proactive about your health and discuss any concerns or symptoms with your healthcare provider.

While these lifestyle changes and preventive measures can help reduce the risk of developing DLBCL and other cancers, it's essential to remember that not all cases of cancer can be prevented. If you have concerns about your risk of DLBCL or other cancers, talk to your healthcare provider for personalized advice and recommendations.

Final words

Understanding diffuse large B-cell lymphoma (DLBCL) and its complexities is essential for timely diagnosis and effective treatment. If you or a loved one has been experiencing symptoms or have concerns about DLBCL, consult a specialist at Max Hospitals for comprehensive evaluation, personalized care, and access to advanced treatment modalities. Our experienced team of oncologists and healthcare professionals is dedicated to providing the highest standard of care, guiding you through every step of your journey towards better health and well-being.