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Diffuse Large B-Cell Lymphoma (DLBCL)

Overview
Treatment Options
Treatments Under Investigation
Clinical Trials
Follow Up
Support
Resources

Overview

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Lymphoma is the most common blood cancer. The two main forms of lymphoma are Hodgkin lymphoma and non-Hodgkin lymphoma (NHL). Lymphoma occurs when cells of the immune system called lymphocytes, a type of white blood cell, grow and multiply uncontrollably. Cancerous lymphocytes can travel to many parts of the body, including the lymph nodes, spleen, bone marrow, blood, or other organs, and form a mass called a tumor. The body has two main types of lymphocytes that can develop into lymphomas: B-lymphocytes (B-cells) and T-lymphocytes (T-cells).

Diffuse large B-cell lymphoma (DLBCL) is the most common form of NHL, accounting for up to 30 percent of newly diagnosed cases in the United States. DLBCL is an aggressive (fast-growing) lymphoma. It can arise in lymph nodes or outside of the lymphatic system, in the gastrointestinal tract, testes, thyroid, skin, breast, bone, or brain. Although DLBCL is the most common form of NHL, there are several subtypes that may affect the prognosis (how well patients will do with standard treatment) and treatment. For example, DLBCL that only affects the brain is called "primary central nervous system lymphoma" and is treated differently than DLBCL that affects areas outside of the brain. Another example is "primary mediastinal B-cell lymphoma" which often occurs in younger patients and grows rapidly in the chest (mediastinum)

Often, the first sign of DLBCL is a painless rapid swelling in the neck, armpit, or groin, which is caused by enlarged lymph nodes. For some patients, the swelling may be painful. Other symptoms include night sweats, unexplained fevers, and weight loss. Most patients with DLBCL are adults, although this lymphoma is sometimes seen in children.

Treatment Options

Because DLBCL advances very quickly, it usually requires immediate treatment. A combination of chemotherapy and the monoclonal antibody rituximab (Rituxan) with or without radiation therapy can lead to a cure in a large number of people with this form of lymphoma.

The most widely used treatment for DLBCL is R-CHOP, which is a mixture of rituximab and several chemotherapy drugs (cyclophosphamide, doxorubicin, vincristine, and prednisone). When treating patients with DLBCL, doctors may also add etoposide (Vepesid), another chemotherapy drug, to R-CHOP, resulting in a drug combination called R-EPOCH.

For many patients, DLBCL does not return after initial treatment; however, for some patients, the disease does return. For patients where the disease becomes refractory (disease does not respond to treatment) or relapses (disease returns after treatment), secondary therapies may be successful in providing another remission or a cure.

A stem cell transplant is the treatment of choice for DLBCL patients whose cancer has returned or relapsed. High-dose chemotherapy coupled with a stem cell transplant can be used to treat patients with DLBCL who have failed initial chemotherapy, but are responsive to a second chemotherapy regimen. The majority of patients undergoing a stem cell transplant will receive their own stem cells (known as an autologous stem cell transplant). Occasionally, a patient will receive stem cells from a donor (known as an allogeneic stem cell transplant). For patients who are not candidates for stem cell transplant, there are various multiagent chemotherapy regimens used for treatment, and there are several clinical trials investigating new combinations of agents.

Treatments Under Investigation

Many treatments are currently being tested in clinical trials for patients with newly diagnosed or relapsed/refractory DLBCL, including:

• Alisertib (MLN8237)
• Bortezomib (Velcade)
• Brentuximab vedotin (Adcetris)
• Everolimus (Afinitor)
• Lenalidomide (Revlimid)
• Panobinostat
• Vorinostat (Zolinza)

Clinical trials are investigating the use of these agents at various treatment stages (first-line, maintenance, etc) and for specific patient populations, including newly diagnosed patients, patients with relapsed or refractory disease, and the elderly. For example, the efficacy and safety of lenalidomide vs the investigator's choice among various agents is currently being investigated in a phase II/III multicenter study of patients with relapsed or refractory disease. In elderly patients, vorinostat in combination with chemotherapy is being assessed as a replacement for an agent with many side effects. The clinical trials investigating these drugs are in various phases of development. It is critical to remember that today’s scientific research is continuously evolving. Treatment options may change as new treatments are discovered and current treatments are improved. Therefore, it is important that patients check with LRF or with their physician for any treatment updates that may have recently emerged.

Clinical Trials

Clinical trials are crucial in identifying effective drugs and determining optimal doses for patients with DLBCL. Patients interested in participating in a clinical trial should talk to their physician or contact LRF's Helpline for an individualized clinical trial search by calling (800) 500-9976 or emailing helpline@lymphoma.org.

Follow Up

Patients in remission should have regular visits with a physician who is familiar with their medical history as well as with the treatments they have received. Some medical tests, such as blood tests and computed axial tomography (CAT) scans, may be required at various times during remission to evaluate the need for additional treatment. Some treatments can cause long-term effects or late effects, which can vary based on duration and frequency of treatments, age, gender, and overall health of each patient at the time of treatment. The doctor will also check for these effects during follow-up care.

Survivors and their caregivers are encouraged to keep copies of all medical records and test results as well as information on the types, amounts, and duration of all treatments received. This documentation will be important for keeping track of any effects resulting from treatment or potential disease recurrences.

Support

A lymphoma diagnosis often triggers a range of feelings and raises many concerns. In addition, cancer treatment can cause physical discomfort. Support groups and online message boards can help patients connect with other people who have lymphoma. One-to-one peer support programs, such as LRF's Lymphoma Support Network, match lymphoma survivors (or caregivers) with volunteers who have gone through similar experiences.

Lymphoma survivors should receive regular medical exams from a physician who is familiar with their medical history as well as the treatments they have received. Survivors and their caregivers are encouraged to keep copies of all medical records and test results as well as information on the types, amounts and duration of all treatments received. This documentation will be important for keeping track of any effects resulting from treatment or potential disease recurrences. For further information, please review our fact sheet on survivorship issues.

Resources

LRF offers a wide range of resources that address treatment options, the latest research advances, and ways to cope with all aspects of lymphoma. LRF also provides many educational activities, from in-person meetings to teleconferences and webcasts. For more information about any of these resources, visit the website at www.lymphoma.org or contact the Helpline at (800) 500-9976 or helpline@lymphoma.org.

Webcasts/Podcasts
Aggressive Non-Hodgkin Lymphomas, Sonali Smith, MD (2012)
Diffuse Large B-Cell Lymphoma, Laurie Sehn, MD (2010)
Teleconferences
Update on Diffuse Large B-Cell Lymphoma, Wednesday, October 5, 2011
In-person Educational Programs
Support Programs
View or order the following publications from our booklets/factsheets webpage:
Diffuse Large B-Cell Lymphoma Fact Sheet

Understanding Non-Hodgkin Lymphoma: A Guide for Patients, Survivors and Loved Ones

Lymphoma Helpline

Professional staff members are available to answer your questions and provide individual support to you and your loved ones. Contact our Helpline, available Monday through Friday from 8:00am - 5:00pm Pacific Standard Time (PST). Call (800) 500-9976 or e-mail.