LYMPHOMA Joginder Singh, MD Medical Oncologist, Mercy Cancer Center Lymphoma is cancer of the lymphatic system. The lymphatic system is made up of organs all over the body that make up and store cells that fight infection and help in immunity. These infection-fighting cells are also called white blood cells. White blood cells are of many kinds neutrophils, lymphocytes, monocytes, eosinophils, and basophils. They differ in ways they fight infections and type of infections they fight. When a patient has lymphoma, lymphocytes become abnormal and grow out of control. These cells can travel to different parts of the body, often abnormal cells collect in small bean-shaped organs called lymph nodes. This causes lymph nodes to swell. The spleen is part of the lymphatic system. Because the lymph system is found all throughout the body, lymphoma can begin anywhere. There are two main types of lymphoma. Hodgkin lymphoma and non-hodgkin lymphoma (NHL). Hodgkin and NHL differ in type of cells they arise. Many people with NHL have B-cell type of NHL (about 85%). The others are T-cell type or an NK-cell type of lymphoma. NHL can be: Slow growing, this is called indolent or low grade. Fast growing, this is called aggressive or high grade. Some people start with slow-growing type of lymphoma that later becomes fast growing. The most common type of non-hodgkin lymphoma in adults is diffuse large cell lymphoma, which is usually aggressive type (sometimes intermediate type) and follicular lymphoma, which is usually the indolent kind. There are treatments for every type of NHL. Some patients with fast-growing NHL can be cured. For patients with slow-growing NHL, treatments may keep the disease in check for many years. This can be true even when tests show that disease remains in some parts of the body.
Diffuse large B-cell lymphoma (DLBC) is the most common type of lymphoma making up 30% of all lymphomas. In the United States, DLBC affects 7 out of 100,000 people each year. It can be fatal if untreated, but with timely and appropriate treatment approximately 2/3 of all patients can be cured. Diffuse large B-cell lymphoma rick factors: Age, gender, and ethnicity affect a person's likelihood of developing DLBC lymphoma. Although diffuse large B-cell lymphoma has been found in people of all age groups, it is found most common in people who are middle aged or elderly. Diffuse large B-cell lymphoma symptoms and signs: A sign is change in body that the doctor sees in an exam or test result. A symptom is a change in body that a patient can see or feel. The most common sign of DLBCL is enlarged growing nonpainful lymph nodes in neck, groin, armpit, or abdomen. Patients may also experience: Fever. Drenching night sweats. Tiredness. Loss of appetite. Weight loss. EXTRANODAL DISEASE In about 40% of patients, DLBCL does not begin in lymph nodes, but instead develops elsewhere, such as stomach, gastrointestinal tract, or other organs. This is called extranodal disease. This may co-exist with nodal disease. ADVANCED VERSUS LOCALIZED DISEASE Most patients (about 60%) are not diagnosed with diffuse large B-cell lymphoma until the disease is advanced (stage III or IV). In the remaining 40% of patients, the disease is confined to one side of the diaphragm (above or below the diaphragm). This is called localized disease. DIAGNOSIS AND STAGING: Diagnosis is usually made by a test called lymph node biopsy. The lymph node biopsy is examined in detail by a pathologist (a doctor who identifies diseases by studying cells and tissues under a microscope. The pathologist is a very essential part of the team because accurate diagnosis is very important to decide the type of treatment. The pathologist may do special stains and other tests at the molecular level to differentiate different types of diffuse large B-cell lymphoma and accurately subtype DLBCL. STAGING TESTS A number of tests may be needed to accurately stage lymphoma. Tests that may be done include: Blood tests. Bone marrow biopsy. PET/CT scan. CT scan (not preferred if combined PET/CT is done.
STAGE GROUPING Staging involves dividing patients into groups (stages) based upon how much of the lymphatic system is involved at the time of diagnosis. Staging helps determine a person's prognosis and treatment options. Stage I: Only one lymph node region is involved. A region means area of lymph nodes and surrounding tissue. Examples include cervical lymph nodes in neck, axillary lymph nodes in armpit, inguinal lymph nodes in groin or mediastinal lymph nodes in chest. Stage II: Two or more lymph node regions or structures on the same side of diaphragm are involved. Stage III Lymph nodes on both sides of the diaphragm are involved. Stage IV: There is widespread involvement of a number of organs or tissues other than lymph node regions or tissues, such as liver, lung, or bone marrow. When a stage is assigned, it also includes letters A or B. The letter B means unexplained fever (higher than 100.4 F. or 38 C), night sweats, or unexplained weight loss. Letter A means these symptoms are absent. TREATMENT OF DIFFUSE LARGE B-CELL LYMPHOMA Treatment of DLBCL depends on whether disease is localized or advanced. In advanced disease, the standard treatment is combination of chemotherapy and immunotherapy. Chemotherapy drugs work by interfering with the ability of rapidly growing cells (like cancer cells) to divide or reproduce themselves. Because most of the other cells in the body do not divide as fast as cancer cells, they are affected less by chemotherapy (chemotherapy kills more cancer cells than normal cells). Moreover, normal cells have the capacity to repair themselves and come back, but cancer cells do not have mechanisms to repair quickly and may not come back. The cells in the bone marrow (blood cells), hair, and lining of gastrointestinal tract grow relatively faster and are more affected by chemotherapy. Immunotherapy uses antibodies that target specific group of cells (usually cancer cells). Rituximab is an antibody that targets B lymphocytes. The most common chemotherapy regimen for advanced DLBCL is called R-CHOP, which includes rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone. The first 4 drugs are given in a vein (IV) on the first day of chemotherapy, while prednisone is given by mouth for the first 5 days of chemotherapy. This regimen is usually repeated every 3 weeks for 6 times in advanced disease. The treatment cycle is given every 3 weeks for cells to recover in between cycles. During this time, patients are closely monitored for signs and symptoms of drug toxicity and side effects.
SIDE EFFECTS OF CHEMOTHERAPY The side effects of R-CHOP chemotherapy that may happen are: Fever and low blood counts, which can be potentially life threatening. Anyone who is getting chemotherapy and who develops a temperature higher than 100.4 F (38 C) should immediately call his or her health care provider. This condition usually requires immediate attention, many times hospitalization and antibodies by vein (IV). Low red blood cell count (anemia) causing weakness, fatigue, and other symptoms and low platelet count causing easy bruising/bleeding. Nausea and vomiting may develop after R-CHOP chemotherapy. Several medications can be given before and after chemotherapy to decrease these side effects. Hypersensitivity reaction can develop, especially to immunotherapy, which most of the time can be managed appropriately. Tumor lysis syndrome. When cancer cells die quickly, they release toxic products into the blood stream, which can cause kidney damage. Preventive treatments are usually given before chemotherapy to reduce the risk of developing tumor lysis syndrome. Other potential complications of chemotherapy include damage to heart (cardiotoxicity), nerves (neurotoxicity), and loss of ability to have children (infertility). These risks as well as ways to monitor them should be discussed with health care provider.
LOCALIZED DISEASE Patients with localized disease are treated with fewer cycles of R-CHOP chemotherapy in combination with radiation therapy to the involved areas. RADIATION THERAPY (RT) Radiation therapy refers to exposure of tumor to high-energy x-rays to slow or stop the growth of tumor. Radiation therapy for lymphoma is given by external beam radiation, meaning that radiation beam is generated by a machine, and exposure to beam typically involves only a few seconds. In general, radiation therapy is given daily, 5 days a week for approximately 3-4 weeks. SURVIVING DIFFUSE LARGE B-CELL LYMPHOMA The chance of surviving DLBCL depends upon many factors. The follow factors are known to reduce the chances of survival. Age older than 60 years. Lactate dehydrogenase (LDH) level higher than normal. LDH is a protein found in blood. The level increases by large amount of cancer cells. Poor general health (performance status). Stage III or IV. More than one involved extranodal disease site. A scoring system known as International Prognosis Index (IPI) gives 1 point for each of the above characteristics for a particular protein score may range from 0 to 5. The higher the score, the less chance of survival. FOLLOWUP AFTER TREATMENT FOR DIFFUSE LARGE B-CELL LYMPHOMA After finishing the planned treatment, the patient should be followed on regular basis to monitor complications of treatment and possible relapse. If there are signs of relapse, biopsy can be done to confirm the diagnosis. The frequency of these visits depends upon comfort of both patient and physician depending upon how aggressive the disease is. The number of CT scans should be limited, particularly in young individuals to limit radiation exposure or risk of second cancers. RECURRENT OR REFRACTORY DIFFUSE LARGE B-CELL LYMPHOMA Recurrent disease is the term used to describe a disease that returns after an initial remission. Refractory disease is a term used to describe a disease that does not fully respond to treatment in the first place. Depending on the person's age and underlying medical problems, treatment may involve a different kind of chemotherapy. If the patient responds to that chemotherapy and is healthy enough, higher dose chemotherapy and a specific kind of bone marrow transplant called autologous hematopoietic stem cell transplantation may be recommended. This type of transplant uses the person's own cells to "rescue" his or her bone marrow from intensive chemotherapy.
CLINICAL TRIALS A clinical trial is an approved research study that is designated to determine if new treatments or improved drugs are better for a patient. A carefully conducted clinical trial may provide best available therapies. WHERE TO GET MORE INFORMATION Your health care provider is the best source of information for questions and concerns related to your medical problem. The following organizations also provide reliable health information: American Cancer Society National Cancer Institute National Library of Medicine Leukemia and Lymphoma Society