Diffuse large B-cell lymphoma (DLBCL) is the most common form of non-Hodgkin’s lymphoma. It is an aggressive cancer of the lymphatic system, but usually responds well to treatment, with a good cure rate. 

This cancer develops from abnormal B-cells that are larger than healthy cells. These unusual B-cells are not clumped together, but are spread out throughout the tumor, or diffuse.

DLBCL usually develops in lymph nodes, but it may also occur elsewhere in the body such as the gastrointestinal tract, skin, testes, eyes, brain, or any other organs. DLBCL that occurs outside of the lymph nodes is referred to as extranodal. In some cases, the abnormal B-cells may be localized in one organ, but they may also be spread throughout the body.

There are several subtypes of DLBCL. Most cases are diagnosed as “not otherwise specified,” but several rare subtypes have been identified. These include T-cell/histiocyte-rich large B-cell lymphoma, EBV-positive DLBCL of the elderly, primary mediastinal large B-cell lymphoma (PMBL), intravascular large B-cell lymphoma, and ALK-positive large B-cell lymphoma.

Development and risk factors

The cause of DLBCL is usually unknown. The disease is most common in people over the age of 50, but it can also occur in children. In general, the risk of developing DLBCL increases with age. Men develop DLBCL slightly more frequently than women. Having a relative who develops DLBCL does not appear to increase a person’s risk of developing the disease.

In some cases, DLBCL may develop from a slow-growing or low-grade lymphoma. There is also some evidence suggesting that it is associated with autoimmune disorders, HIV infection, or organ transplant.

Symptoms and diagnosis

Typical symptoms of DLBCL are lymph node swelling, usually in the neck, armpit, or groin, fatigue, weight loss, fevers, and night sweats.

Depending on where DLBCL develops, symptoms may vary. For example, if it develops in the gastrointestinal tract, it may cause abdominal pain or diarrhea. Because it causes swelling of the affected tissues, it can cause pain in affected areas.

Doctors typically need to do a biopsy to diagnose DLBCL. During this procedure, a small sample of the affected tissue is removed and sent for a microscopic examination by a pathologist who can identify abnormal cells. If abnormal cells are present, additional tests may be done to determine the type of lymphoma. Sometimes these can be done from the original biopsy sample, but occasionally a second biopsy is necessary.

Blood tests, CT scans, or bone marrow tests may also be done to determine whether the DLBCL has spread to other areas of the body. These tests help doctors determine the stage of the disease. Depending on how many areas of the body are affected, DLBCL is rated from stage 1 (one group of lymph nodes affected) to stage 4 (several organs affected).

Treatment and prognosis 

Because DLBCL is a fast-growing form of lymphoma, it needs immediate treatment. The disease is usually treated with a combination of chemotherapy, monoclonal antibodies, and steroids. Radiation therapy is also sometimes used.

The most common therapy combination is called R-CHOP,  which stands for rituximab (Rituxan), cyclophosphamide (Cytoxan), hydroxydaunomycin, vincristine (Oncovin), and prednisolone. These medications are given as an injection about every three weeks. Treatment usually lasts several months.

Patients who are not responsive to these therapies, or those who experience a relapse of DLBCL, might be treated with a stem cell transplant in combination with high-dose chemotherapy.

DLBCL usually responds well to treatment, but the prognosis depends on the patient’s general health and the stage of the disease. In general, early-stage cases of DLBCL are more likely to go into remission than late-stage cases.

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