DLBCL-primary mediastinal B-cell lymphoma

Diffuse Large B-Cell Lymphoma (DLBCL) 

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. 

Source: Lymphoma Research Foundation