Lymphoma is a cancer that starts in cells called lymphocytes, white blood cells that make up the immune system and protect the body from germs and other health dangers.
B lymphocytes (B-cells) produce proteins called antibodies that fight and kill germs and prevent them from infecting the body.
T lymphocytes (T-cells) have different roles—some attack and kill abnormal cells, while others regulate how various immune system cells operate.
B-cell lymphoma is the most common type of blood cancer in western countries, according to the American Society of Clinical Oncology (ASCO), with about 90 percent of all lymphoma cases being B-cell lymphomas.
Among B-cell lymphomas, the majority are non-Hodgkin lymphomas (NHL). In the United States, B-cell lymphomas account for about 85 percent of all non-Hodgkin lymphomas, according to the American Cancer Society (ACS). Hodgkin lymphoma may also affect B-cells.
B-cell lymphoma symptoms
Certain symptoms, called B symptoms, are associated with B-cell lymphomas, including:
Fever
Night sweats
Unintentional weight loss of more than 10 percent of body weight over six months
Not all people with B-cell lymphomas experience B symptoms, however, and some types take longer to start causing symptoms than others.
Causes of B-cell lymphoma
Although lymphomas develop when certain types of genetic mutations arise in white blood cells, it’s usually unclear what causes this to happen. Most cases of lymphoma can’t be traced to a specific cause. However, scientists have identified several risk factors associated with an increased risk of B-cell lymphoma, including:
Older age
Chronic infections
Immune system deficiencies or conditions, such as autoimmune disorders
Types of B-cell lymphomas
There are many different types of B-cell lymphomas, including:
The most common type of lymphoma, DLBCL, makes up about 33 percent of all NHL cases in the United States, according to the ACS. Most DLBCL patients are diagnosed in their mid-60s.
The cancer typically starts within a lymph node in the chest, abdomen, neck or armpit. If the mass forms in the neck or armpit, it’s easier to feel or see. Less commonly, DLBCL may start in the intestines, bones, brain or spinal cord.
Diffuse large B-cell lymphoma symptoms
The first apparent symptom of DLBCL may be swelling in the lymph nodes of the neck, underarms or groin. The swelling may be painless or painful, depending on the patient.
Other potential symptoms include:
Night sweats
Fever
Unintentional weight loss
Tiredness
Poor appetite
Shortness of breath
DLBCL is diagnosed with a biopsy, which involves a surgical procedure to remove a small sample of the potentially cancerous area. The sample is sent to a laboratory, where it’s inspected under a microscope for cancer cells. In addition to a biopsy, other diagnostic tests such as imaging scans may be used to determine whether and where the cancer may have spread.
The cancer tends to quickly grow and spread to organs beyond just the lymph nodes. With treatment, however, about two out of every three people with the cancer are cured, according to ASCO.
Diffuse large B-cell lymphoma treatment
The standard treatment regimen for DLBCL is called R-CHOP, which combines an immunotherapy drug called Rituxan® (rituximab) with four chemotherapy drugs (cyclophosphamide, doxorubicin, vincristine and prednisone, or CHOP). The combination is given in multiple cycles every three weeks. Patients with heart problems may be treated with different chemotherapy drugs because doxorubicin may cause heart damage. Radiation therapy may also be used if the cancer is contained within a small area.
Primary mediastinal large B-cell lymphoma is a subtype of DLBCL that primarily affects women between the ages of 30 and 40. According to the ASCO, it accounts for about 2.5 percent of all NHL cases. In this type of DLBCL, the cancer appears as a large mass in the mediastinum, which is located behind the breastbone, in the middle of the chest. Since the cancer can quickly grow large, it may start to press down on important areas—such as the superior vena cava (a major vein)—and affect blood flow or breathing. Like other types of DLBCL, it tends to be responsive to treatment. The R-CHOP treatment regimen is commonly used.
Diffuse large B-cell lymphoma survival rates
Five-year relative survival rates estimate how likely it is that a person with a specific cancer will be alive five years after diagnosis, compared with people who don’t have that cancer. These numbers are only group estimates, and they don’t account for all of the many individual factors that influence outcomes.
The ACS gives the overall five-year relative survival rate for DLBCL as 64 percent, based on cancer patients diagnosed between 2011 and 2017. In other words, people with DLBCL are, on average, about 64 percent as likely as people without DLBCL to live five years or longer after their initial diagnosis.
The ACS also supplies survival rates for each of these three stages: localized (the cancer hasn’t spread beyond one area), regional (the cancer has spread to nearby areas) and distant (the cancer has spread to distant body parts).
Below are the five-year relative survival rates for DLBCL, according to the cancer’s stage.
Localized: 74 percent
Regional: 73 percent
Distant: 57 percent
Follicular lymphoma
The second most common type of lymphoma in the United States is follicular lymphoma, which accounts for about 20 percent of all NHL cases, according to the ACS. It’s typically slow-growing and starts to form in lymph nodes.
Follicular lymphoma symptoms
Common symptoms of follicular lymphoma include:
Swelling in the neck, underarms, abdomen or groin (due to lymph node enlargement) may be a sign of follicular lymphoma.
Fatigue is common among follicular lymphoma patients.
Standard procedures are used to diagnose follicular lymphoma, including a biopsy of the potentially cancerous area and imaging tests to assess the cancer’s location.
Follicular lymphoma treatments
Radiation therapy may be a treatment for early-stage follicular lymphoma. As the cancer progresses, treatment options also include chemotherapy and targeted therapy. Some patients may not require immediate treatment but rather a watchful waiting approach, where symptoms are monitored.
If the follicular lymphoma transforms into DLBCL, the treatment mimics that for traditional DLBCL.
Follicular lymphoma survival rate
The overall five-year relative survival rate for follicular lymphoma is 90 percent, based on cases between 2011 and 2017, according to the ACS. Patients with follicular lymphoma are, on average, about 90 percent as likely as people without follicular lymphoma to live five years or longer after their initial diagnosis.
Below are the five-year relative survival rates for follicular lymphoma based on the cancer’s stage.
Localized: 97 percent
Regional: 91 percent
Distant: 86 percent
Mantle cell lymphoma
Mantle cell lymphoma (MCL) accounts for about 5 percent of NHL cases, according to the ACS. Men and older adults (above age 60) are most at risk. The cancer may affect various organs and areas of the body, including the bone marrow, lymph nodes, spleen and digestive tract.
Symptoms may include:
Poor appetite
Unintentional weight loss
Fever
Night sweats
Stomach problems
Discomfort due to swelling in the tonsils, liver or spleen
Lower back pain
Fatigue
Diagnosis relies on a surgical biopsy to remove a sample of the potentially cancerous area (usually from a lymph node).
The cancer may be slow- or fast-growing. If it’s slow-growing, it may not need immediate treatment, and patients are monitored for symptoms and other complications before moving on to treatment.
A combination of chemotherapy and targeted therapy is a common treatment regimen for mantle cell lymphoma. Young, healthy patients may be given a strong cycle of chemotherapy along with a bone marrow transplant, followed by immunotherapy to prevent cancer from coming back (maintenance therapy). This regimen tends to give patients the best chance of maintaining a long remission. Older patients or those with other health complications may not be able to withstand a bone marrow transplant, but may still receive the other components of the treatment regimen, including maintenance therapy with an immunotherapy drug.
Small lymphocytic lymphoma (SLL)
Small lymphocytic lymphoma is similar to chronic lymphocytic leukemia (CLL), a common type of leukemia. Both produce cells called small lymphocytes—but in CLL, the cancer primarily develops in the blood and bone marrow, while SLL causes cancer to grow in the lymph nodes and spleen. SLL accounts for about 5 percent of all NHL cases, according to ASCO.
Symptoms may not be apparent until later in the disease’s progression. When they do occur, symptoms may include:
Tiredness
Shortness of breath
Swelling in the neck, armpit, groin or abdomen
Fever
Unintentional weight loss
Night sweats
Frequent infections in areas such as the skin and lungs
For diagnosis, doctors may do blood tests and a bone marrow aspiration and biopsy, which involve removing bone marrow and sometimes a small piece of bone to check for cancerous cells.
SLL tends to be slow-growing and may not require immediate treatment. Patients need to be monitored, and treatment may be initiated if symptoms or other problems appear.
The treatment options for SLL and CLL are the same. A common regimen involves chemotherapy with targeted therapy. One combination that may be used as a first treatment is the targeted therapy drug Imbruvica® (ibrutinib) along with the monoclonal antibodies Gazyva® (obinutuzumab) or Rituxan®. Alternatively, targeted therapy drug Venclexta (venetoclax) may be given along with Gazyva or Rituxan.
Double hit and triple hit lymphomas
About 5 percent of lymphomas are subtypes called double hit lymphomas or triple hit lymphomas, according to the ASCO. These subtypes tend to affect older adults.
It’s called “double hit” if the cancer cells have mutations on two particular genes (MYC gene and either the BCL2 or BCL6 gene). Cancer cells with mutations present on all three of these genes are called “triple hit.”
Double hit and triple hit lymphomas are aggressive and have a high risk of spreading to parts of the central nervous system, such as the brain and spinal cord.
Symptoms for double hit and triple hit lymphomas include:
Swelling
Night sweats
Fever
Unintentional weight loss
Tiredness
Poor appetite
Shortness of breath
Diagnosis will likely involve standard procedures such as a biopsy, but more extensive analysis of the cancer cells is needed to determine whether the genetic mutations are consistent with a double hit or triple hit lymphoma.
Currently, there’s no standard treatment for this type of lymphoma, as it tends to not respond as well to the established regimens as other subtypes. However, chemotherapy and Rituxan are commonly used.
Marginal zone lymphomas
Marginal zone lymphomas are slow-growing and characterized by cancer cells that appear small under a microscope. According to the ACS, 5 percent to 10 percent of lymphomas are marginal zone lymphomas. Within this group, there are three subtypes.
Extranodal marginal zone B-cell lymphoma or mucosa-associated lymphoid tissue (MALT) lymphoma: The most common type of marginal zone lymphoma, MALT lymphomas don’t start in lymph nodes, which is why they’re called extranodal. A MALT lymphoma that begins in the stomach may be due to a type of bacteria (Helicobacter pylori). In this case, the cancer may be treated and cured with antibiotics. MALT lymphomas may also begin in the lungs, skin, bowels, thyroid, salivary glands or near the eye. Having an autoimmune disease, including lupus or rheumatoid arthritis, may increase the risk of getting this type of lymphoma. Radiation therapy may be used if the cancer is limited to one organ. Rituxan with or without chemotherapy may also be a treatment option.
Splenic marginal zone B-cell lymphoma: This rare type of marginal zone lymphoma typically starts in the spleen and spreads to the blood and bone marrow. As the cancer causes swelling in the spleen, symptoms may include fatigue and discomfort in the upper abdomen. This cancer tends to be slow-growing and may not need treatment until symptoms appear. Treatment options may include Rituxan, possibly with chemotherapy. This cancer has been linked to hepatitis C. If a patient has both, sometimes treating the viral infection helps with the cancer.
Nodal marginal zone B-cell lymphoma: This is another rare type of cancer, accounting for about 1 percent of all lymphomas, according to ASCO. It tends to begin in the lymph nodes and remain there, though it sometimes spreads to the bone marrow. Treatment options are similar to those used to treat follicular lymphoma: radiation alone for early-stage disease, or chemotherapy, targeted therapy and/or radiation therapy as the cancer progresses.
Diagnosing marginal lymphomas involves standard procedures such as a biopsy and imaging tests.
Symptoms may depend on the location of the cancer. For example, MALT lymphomas involving the digestive system may cause symptoms such as nausea, vomiting, abdominal pain, indigestion and gastric bleeding. B symptoms—such as fever, night sweats and weight loss—may also be present.
About 1-2 percent of lymphomas are lymphoplasmacytic lymphoma, according to ASCO. This cancer is slow-growing and causes small cancer cells to form in areas such as the bone marrow, lymph nodes and spleen. Often, this cancer causes the body to produce excessive amounts of a protein called an M protein. In this case, the cancer is called Waldenstrom’s macroglobulinemia (WM).
Overproduction of this protein thickens the consistency of the blood, leading to symptoms such as:
Headaches
Blurry vision
Dizziness
Shortness of breath
B symptoms may also occur, such as:
Weight loss
Fever
Night sweats
Diagnosis may involve blood and other tests to measure the patient’s M protein levels, as well as a biopsy to confirm the diagnosis.
WM may take years to start causing symptoms, and treatment may be delayed until problems arise. Treatment options include chemotherapy, targeted therapy or a combination of the two.
Burkitt lymphoma
Burkitt lymphoma is a fast-growing cancer that requires immediate treatment. It accounts for 1 percent to 2 percent of lymphomas in adults, according to the ACS, but it’s more common in children. Males are more at risk than females.
The three subtypes of Burkitt lymphoma are:
Endemic Burkitt lymphoma most often occurs in Africa and is rare in the United States. It tends to occur in people who have been infected with the Epstein-Barr virus, the virus behind mononucleosis or “mono.”
Sporadic or nonendemic Burkitt lymphoma tends to cause a large tumor in the abdominal area or the ovaries, testicles and other organs. This type is more common in the United States than the endemic type.
Immunodeficiency-related Burkitt lymphoma occurs in patients with weak immune systems. People who have HIV or AIDS or have undergone organ transplantation may be more at risk.
Lymph node swelling in the head and neck may be the first sign of Burkitt lymphoma. This cancer also may include B symptoms, such as:
Fever
Night sweats
Weight loss
Diagnosis involves blood tests, a biopsy of a lymph node and/or bone marrow, and other imaging scans.
Aggressive, high-dose chemotherapy is a common treatment option. With chemotherapy, more than 80 percent of patients are estimated to achieve long-term remission, according to ASCO.
Primary central nervous system (CNS) lymphoma
CNS lymphoma causes cancer in the brain or spinal cord and often spreads throughout the central nervous system. Older adults and people with deficient immune systems, such as AIDS patients, are more at risk, but the cancer is rare.
Symptoms may include:
Headaches
Confusion
Vision problems
Weakness in the limbs
Depending on the cancer’s stage and location, treatment may involve chemotherapy, corticosteroid drugs, radiation therapy or a combination of those options.
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