Diffuse Large B-Cell Lymphoma (DLBCL): Understanding the Basics

What is Large B-Cell Lymphoma?

Diffuse large B-cell lymphoma (DLBCL) is a form of blood cancer. It begins in B lymphocytes, a kind of white blood cell. These cells are crucial for the immune system, helping protect the body from infections. In DLBCL, B cells multiply in a disorderly way.

DLBCL is a variation of non-Hodgkin lymphoma and stands out as the most common. This lymphoma type is aggressive, meaning it develops quickly. The name “diffuse large B-cell lymphoma” describes how it appears under a microscope: the B cells are larger than usual and spread out rather than clumped.

Symptoms might include swelling of lymph nodes, fever, weight loss, and night sweats. Early diagnosis and treatment are key to managing this condition, so anyone experiencing unusual symptoms should consult a healthcare professional.

Signs of DLBCL

People with DLBCL often notice painless lumps. These bumps usually appear in the neck, armpit, groin, or abdomen. Men might find them in the testicles. These lumps are swollen lymph nodes that can grow quickly. Sometimes, you can’t feel these lumps because they are deep inside the body. This is known as ‘bulky disease.’ When DLBCL affects areas outside of lymph nodes, it is called ‘extranodal’ disease. This happens in about 4 out of 10 people with DLBCL.

The exact signs someone experiences can change depending on where the DLBCL is in the body. When it appears in places like the stomach or bowels, it might cause discomfort or pain. Other signs include feeling sick, having diarrhea, or even bleeding. About 1 in 4 people find it affecting their bowels.

If DLBCL is in the chest, it might lead to a cough or breathlessness. Lymphoma in the lungs is less common, affecting fewer than 1 in 10 people.

Another set of signs called ‘B symptoms’ affects around 1 in 3 people with DLBCL. They might experience fevers, night sweats, and unexpected weight loss. Feeling tired and having a loss of appetite are also common.

Who is Affected by DLBCL?

Diffuse Large B-Cell Lymphoma (DLBCL) is mostly seen in individuals aged 65 and above. Interestingly, men have a slightly higher chance of being diagnosed with DLBCL compared to women.

Causes of DLBCL:

  • Often unknown
  • Sometimes linked to immune system issues

Related Conditions:

  • Autoimmune diseases: Conditions like rheumatoid arthritis or lupus can lead to long-term inflammation, potentially increasing DLBCL risk.
  • HIV: Individuals with this virus might also face a higher risk.
  • Organ transplants: People who have undergone such procedures may see an association with DLBCL.

In some cases, DLBCL develops from a previous low-grade lymphoma. This transformation occurs when a slow-growing lymphoma changes into a more aggressive form.

Types of DLBCL

Uncommon Forms of DLBCL and Other Significant B-Cell Lymphomas

There are several less common types of DLBCL and other large B-cell lymphomas. These subtypes can cause symptoms that might be different from the more common types but often receive similar treatments.

  • Primary Mediastinal Large B-Cell Lymphoma (PMBL):
    This type usually affects individuals in their 20s and 30s and is more frequent among women. It originates from B cells in the thymus, a small gland found behind the breastbone. PMBL typically appears as a noticeable mass in the chest. Although it can extend to lymph nodes, it seldom spreads to other areas of the body. Symptoms include trouble breathing, coughing, difficulty swallowing, swelling in the neck and face, headaches, and dizziness. Treatment often combines therapies used for DLBCL with additional chest-area radiotherapy. Occasionally, doctors might suggest more intensive treatments or participation in clinical trials.
  • T-Cell/Histiocyte-Rich Large B-Cell Lymphoma:
    This variety is recognized by the unique cell types visible under a microscope. It appears more frequently in middle-aged men but can occur at any age. Common signs include enlarged lymph nodes, spleen or liver swelling leading to abdominal discomfort, and feeling generally unwell, often with B symptoms such as fever, night sweats, and weight loss. Accurate diagnosis is crucial to receiving the best treatment, which usually aligns with treatments for the standard type of DLBCL.
  • EBV-Positive DLBCL Not Otherwise Specified:
    Found mostly in people over 50, this subtype connects to the Epstein–Barr virus (EBV). Symptoms rely on the affected body part. Most individuals with this form (70%) experience lymphoma outside lymph nodes, commonly affecting the skin, lungs, tonsils, or stomach. Around 30% have lymph node-only involvement. The treatment protocol remains similar to that for DLBCL NOS.
  • Intravascular Large B-Cell Lymphoma:
    Predominantly affecting older adults, this subtype is marked by abnormal lymphocytes within the bloodstream, located within tiny blood vessels known as capillaries. Enlarged lymph nodes are uncommon. Symptoms vary with affected locations, including confusion, seizures, dizziness, skin patches or lumps, B symptoms, and potential liver or spleen swelling. Treatment generally mirrors the approach for DLBCL NOS.

Treatments for DLBCL

Managing Early-Stage DLBCL

For those dealing with early-stage diffuse large B-cell lymphoma (DLBCL), typically stage 1 or stage 2, the main approach involves a mix of chemotherapy and antibody therapy, followed by radiotherapy. One of the most common chemotherapy protocols used is R-CHOP, which consists of five medications: rituximab, cyclophosphamide, doxorubicin (often called hydroxydaunorubicin), vincristine (also known as Oncovin®), and prednisolone. Patients receive these drugs typically through a vein, but prednisolone is taken orally for five days.

Treatment is arranged in cycles, each lasting three weeks with a short recovery period after each cycle, allowing the body time to rest. The number of cycles a patient undergoes can vary, but many complete three to four before moving on to radiotherapy. When radiotherapy isn’t suitable, more cycles of R-CHOP might be needed. Radiotherapy may be skipped in cases where the patient is low-risk based on certain factors such as age, fitness, and lab results or if the disease shows complete response to R-CHOP, which is often the case for some individuals.

In some cases, additional medication called a growth factor is provided. These are injections given under the skin to help reduce the chance of infection, administered either by nurses or self-administered at home. After the prescribed treatment rounds of R-CHOP, specific radiotherapy tailored to the affected area might be recommended, especially when the disease area is larger. This decision relies heavily on the individual’s circumstances, and healthcare providers ensure patients are informed of the risks and benefits involved.

Approaches for Advanced-Stage DLBCL

For stages 3 and 4 of DLBCL, a frequent treatment involves Pola-R-CHP, combining polatuzumab vedotin with traditional chemotherapy drugs like rituximab, cyclophosphamide, doxorubicin, and prednisolone. Polatuzumab vedotin, a unique medication, acts by attaching to proteins on B cells, delivering potent drugs directly to the cancer cells.

Other available treatments include the standard R-CHOP cycles. Adjusted doses or alternative treatments may be considered when full-strength chemotherapy isn’t feasible due to health reasons. Adjusted regimens like reduced-dose Pola-R-CHP or R-CHOP, and RGCVP are used particularly among individuals with heart concerns. Participation in clinical trials might also be an option.

Patients at high risk for relapse or dealing with particularly aggressive types of DLBCL may need more intense treatment. These stronger interventions, though more demanding, include intensive procedures like R-CODOX-M/R-IVAC and DA-EPOCH-R. These treatments balance out the need for effectiveness while maintaining awareness of increased side effects. Most people with advanced stages do not receive further radiotherapy, unless specific situations arise, like the presence of residual lymphoma in one body area or prevention of recurrence if the disease area was initially large.

Preventing CNS Involvement in DLBCL

Around 5% of DLBCL cases risk recurrence in the central nervous system (CNS), which poses a significant challenge. When a physician suspects a patient might be vulnerable to CNS involvement, preventive treatment called CNS prophylaxis is considered. This doesn’t apply to everyone, as most won’t need this extra treatment measure. For those who are advised to pursue CNS prophylaxis, this approach aims to preemptively address potential issues within the brain and spinal cord areas.

A Group Of Diverse Silhouettes Representing Different Age Groups And Genders, Standing Together To Symbolize The Wide Range Of People Affected By Dlbcl

DLBCL Outlooks

Diffuse Large B-Cell Lymphoma (DLBCL) is mostly treated with the goal of eliminating the disease. Many patients respond positively to treatment, achieving complete remission with no signs of lymphoma.

The future of each person’s condition varies. It hinges on factors like the stage of lymphoma, the specific DLBCL type, and the person’s overall health. It’s essential to consult with a lymphoma specialist. They assess test results and personal factors such as age and physical fitness to estimate the potential response to treatment.

Survival rates are often perplexing and might not reflect an individual’s circumstances. These statistics represent how a group fared over time and don’t apply to personal situations directly. Treatments are continually advancing, and survival rates usually track 5 to 10 years after therapy. These numbers show past outcomes, and those patients might not have received current treatments. This is why many find these statistics not particularly useful.

For a broader look at survival information regarding DLBCL, the Cancer Research UK website has materials that could be helpful. Yet, always emphasize personal consultation with healthcare professionals for the most accurate guidance.