Lymphoma is a cancer of lymphocytes, which are white blood cells of the immune system that are located in the bone marrow, thymus, spleen, and lymph nodes throughout the body. Lymphoma is divided into two main subtypes: Hodgkin lymphoma (HL) and Non-Hodgkin Lymphoma (NHL). Hodgkin Lymphoma is a separate disease and is discussed elsewhere. Non-Hodgkin lymphoma is a general term used for more than 30 sub-types of lymphoma. Diffuse Large B-cell Lymphoma (DLBCL) is the most common subtype of NHL, affecting about 7 out of 100,000 people each year. The disease most commonly affects people who are middle-aged or elderly, and men are slightly more likely to develop DLBCL than women. In the United States, Caucasians have a higher rate of DLBCL than Asians or African Americans.
DLBCL is a more aggressive form of lymphoma that can develop in either lymph nodes or outside of the lymphatic system, in the gastrointestinal tract, testes, thyroid, skin, breast, bone or brain.
Symptoms of Diffuse Large B-cell Lymphoma
The first sign of DLBCL is often a fast-growing mass or swelling in the neck, groin, or abdomen. Patients may also experience fever, weight loss, or drenching night sweats (“B-symptoms”). In approximately 40% of cases, the cancer begins outside of the lymph nodes. In this case, the most common location is in the stomach or gastrointestinal tract. These patients may have abdominal pain or trouble with bowel movements (either diarrhea or constipation).
Risk Factors for Diffuse Large B-cell Lymphoma
Age, gender, and ethnicity are the most prominent risk factors affecting a person’s risk of developing DLBCL. As mentioned earlier, men and Caucasians have a higher risk for developing the disease. The average age of people with DLBCL is approximately 64 years old. There are no environmental toxins that have been confirmed to increase the risk of DLBCL.
How Diffuse Large B-cell Lymphoma is Diagnosed
After presenting to either their primary care physician or other healthcare provider with the symptoms described above, patients are usually referred to a surgeon for a biopsy. DLBCL is confirmed by removing all or part of an enlarged lymph node or mass. In most cases the biopsy is taken from an enlarged node that is close to the skin’s surface, such as in the neck, under the arm or in the groin. If there are no suitable areas close to the skin, biopsy of a deeper lymph node can be done under general anesthesia. Once the biopsy is done, the tissue is taken to the lab to be processed. Based on how the cells appear under the microscope and several other tissue exam techniques, a pathologist makes the diagnosis of DLBCL.
Once the diagnosis is confirmed, patients will often be sent for further testing. Prior to starting treatment, patients will need blood tests, CT scans of the neck, chest, abdomen, and pelvis, if they were not done initially, testing of heart function with either an echocardiogram or MUGA scan, and sometimes often a PET Scan is also needed. A PET scan is similar to a CT scan, but can give the doctor additional information in determining the extent of disease and its response to treatment. The CT scan may still show some enlarged lymph nodes due to scar tissue but the PET scan is able to tell whether the lymph nodes are still affected, and also serves as a point of comparison to measure how well a patient is responding to treatment. In addition, all patients require an initial bone marrow biopsy to rule out bone marrow involvement with DLBCL.
DLBCL is divided into four stages based on the extent of lymph nodes involved with disease. The current staging system used is called the Ann-Arbor staging system. The system is as follows:
• Stage I – Involvement of one lymph node region (I), or a single non-lymphatic organ or site (IeE).
• Stage II – Involvement of two or more lymph node regions on the same side of the diaphragm (II) or of one lymph node region and an adjacent non-lymphatic organ or site (IIe).
• Stage III - Involvement of lymph node regions on both sides of the diaphragm, which may include the spleen (IIIs) and/or a non-lymphatic organ or site (IIIe, IIIes).
• Stage IV - Involvement of one or more non-lymphatic organs.
Each stage also includes a letter, A or B, to indicate whether fever, weight loss, or night sweats are present. "A" means these symptoms are not present; "B" means they are.
Treatment Options for Diffuse Large B-cell Lymphoma at Tufts Medical Center
Depending on the extent of disease, treatment will include either radiation therapy, chemotherapy, or both. The most common chemotherapy regimen used to treat DLBCL is termed “R-CHOP” which is an acronym standing for a combination of five drugs: rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone. The first four drugs are given by an IV infusion (given by vein) over the course of several hours, and are given in our infusion center on South 8. Prednisone is taken by mouth once daily for five days, starting on the day of IV chemotherapy. Admission to the hospital is not usually required for this treatment. Based on the extent of disease present, some patients require radiation therapy, which is also available.
If a patient relapses or does not respond to initial therapy, another course of chemotherapy is recommended at the time of relapse. There are various combinations of chemotherapy drugs that can be used in this situation, and the choice of regimen varies depending on each patient’s medical history. If a patient responds well to a second course of chemotherapy and meets other specified medical criteria, autologous stem cell transplant (transplant using a patient’s own bone marrow cells) provides the best option for cure. If a patient relapses again after autologous stem cell transplant, further options include more chemotherapy and possibly an allogeneic bone marrow transplant (transplant of bone marrow cells from a matched relative or matched unrelated donor).
All of the above mentioned therapies are available and commonly performed at Tufts Medical Center.
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