What Is B-Cell Lymphoma?

Updated on 2024-01-28 15:21:34 0 0

By Corey Whelan

Medically reviewed by Gagandeep Brar, MD

B-cell lymphoma is a type of non-Hodgkin's lymphoma (NHL). NHL is a cancer that starts in white blood cells called lymphocytes. Most non-Hodgkin's lymphomas in the United States are B-cell lymphomas.1

American Cancer Society. Types of B-cell lymphoma.

B-cell lymphoma starts in B lymphocytes (B cells), a type of white blood cell that makes antibodies (proteins that are part of the immune reaction, especially to fight infection). B lymphocytes are an integral part of the immune system. They’re produced by stem cells located in the bone marrow.

There are several types of B-cell lymphoma. This article will go over the most common types, as well as several rarer forms. It will also discuss symptoms and treatments you can expect, as well as prognosis and coping with this disease.

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Types of B-Cell Lymphoma

Diffuse large B-cell lymphoma (DLBCL) is the most common type of B-cell lymphoma in the United States and worldwide. It has several subtypes.2 Follicular lymphoma is another common type.1 There are also several rare types and subtypes of this disease.

The type you have will be determined in part by the appearance of the malignant cells under a microscope.

B-cell lymphomas can be either indolent or aggressive. Indolent lymphomas grow slowly, while aggressive lymphomas may be more likely to spread quickly. Indolent lymphomas may turn into aggressive lymphomas over time. Both indolent and aggressive lymphomas may respond well to treatment.

Diffuse Large B-Cell Lymphoma (DLBCL)

In the United States, approximately 1 out of 3 cases of non-Hodgkin's lymphoma are of diffuse large B-cell lymphoma.1 In this type, the B cells have a large appearance when viewed under a microscope.

DLBCL can occur at any age, including in children. However, it's most common in older adults. Most people who get this disease are over 60 years of age.2 DLBCL is an aggressive form of B-cell lymphoma.

It can develop in or around the lymph nodes and also in any organ of the body, including the gastrointestinal tract and the brain. Even though it's aggressive, it often responds well to treatment and can, potentially, be cured.2

DLBCL has several subtypes. They include:1

Primary mediastinal B-cell lymphoma: This lymphoma starts in the middle of the chest behind the breastbone (mediastinum). It's an aggressive form of lymphoma and can become large quickly, pressing on the windpipe and making it hard to breathe. It is most diagnosed in young women.1 Even though this type of lymphoma is aggressive, it often responds well to treatment.1
Primary DLBCL of the central nervous system: This subtype is comprised of all DLBCLs that start in the eyes or the brain.
Primary cutaneous DLBCL, leg type: This subtype can occur in several parts of the body, including the legs, arms, buttocks, and trunk. Under a microscope, the malignant B cells look large and red, or bluish red.
T-cell/histiocyte-rich B-cell lymphoma: This rare, aggressive subtype has less than 10% abnormal B cells and a large amount of T cells, another type of white blood cell.3
Epstein-Barr virus (EBV)-positive DLBCL of the elderly: This subtype is most common in people over 50.2 It occurs in people who test positive for the Epstein-Barr virus. EBV is a member of the herpesvirus family.
DLBCL not otherwise specified (NOS): Any DLBCL that doesn't fall into any of these subtypes is considered NOS—not overwise specified. In the U.S. and other developed countries, 25%–30% of non-Hodgkin lymphomas fall into this category.2

Follicular Lymphoma

Follicular lymphoma accounts for 1 in 5 cases of lymphoma in the United States.1 This type of cancer usually affects people over age 60.

Follicular lymphomas may grow in lymph node sites throughout the body and in the bone marrow. It's not considered to be a curable condition. However, follicular lymphoma does respond well to treatment. People can live many years with this disease.4

Most follicular lymphomas are indolent, but some are aggressive and fast growing. In some instances, follicular lymphomas may turn into aggressive, diffuse large B-cell lymphomas.

Mantle Cell Lymphoma

Mantle cell lymphoma is more commonly found in males than in females.1 It usually occurs in males aged 60–70.5

Mantle cell lymphoma develops from malignant B cells located in an area of the lymph nodes called the mantle zone. Around 5% of lymphomas are mantle cell lymphomas.1

Mantle cell lymphoma can be difficult to treat. However, treatments including new medications and stem cell transplants have shown significant promise for treating this disease.6

Marginal Zone Lymphomas

Marginal zone lymphomas are slow-growing cancers categorized by cancer cells that look small under a microscope. The three subtypes are:

Extranodal marginal zone B-cell lymphoma: Also called mucosa-associated lymphoid tissue (MALT) lymphoma, this type originates outside the lymph nodes. They may be gastric (in the stomach) or non-gastric. Gastric MALT lymphomas may be caused by infection with a type of bacteria called Helicobacter pylori. In some instances, treating this infection may also cure the cancer.1
Nodal marginal zone B-cell lymphoma: This rare disease is slow growing and usually remains in the lymph nodes. In some instances, cancerous cells may also be found in the bone marrow.
Splenic marginal zone B-cell lymphoma: This rare disease affects the spleen, bone marrow, and blood. It is sometimes linked to hepatitis C infection.1

Burkitt Lymphoma

Burkitt lymphoma is a fast-growing cancer that is earmarked by medium-sized cancer cells. It is more common in children than in adults.1 It’s also more likely to affect male children than female children.1

There are several types of Burkitt lymphoma. The type most common in the United States is sporadic Burkitt lymphoma.1 Sporadic Burkitt lymphoma often starts as a large tumor in the abdomen. In some instances, this condition is linked to the Epstein-Barr virus.7

When Burkitt lymphoma is diagnosed and treated early with chemotherapy, it has a cure rate of around 50%.1

Other types of this condition include endemic Burkitt lymphoma and immunodeficiency-related Burkitt lymphoma.

Primary Central Nervous System Lymphoma (PCNSL)

This cancer develops in the brain and/or in the spinal cord. Unlike other forms of lymphoma, PCNSL may cause symptoms like confusion and changes in language and behavior.8 It may also cause vision problems.

PCNSL affects around 1,500 people annually in the United States. It is most common in people who are immunocompromised, especially those with human immunodeficiency virus (HIV). It is also found in older individuals.

Primary Intraocular Lymphoma (Eye Lymphoma)

Primary intraocular lymphoma starts in the eyeball. People with primary central nervous system lymphoma may also present with this condition. It is the second most common form of eye cancer in adults.1

Common Symptoms of B-Cell Lymphoma

B-cell lymphoma symptoms vary, based on the type or subtype you have. Some common symptoms include:9

Enlarged spleen
Painless, swollen lymph nodes
Profuse night sweats
High fever that comes and goes with no apparent cause
Significant, unexplained weight loss of around 10% of your body weight

Based on the location of the tumor, you may also experience trouble breathing, or pain in your chest, back, or stomach. Gastrointestinal symptoms like diarrhea can also occur.

Causes of B-Cell Lymphoma

B-cell lymphoma occurs when lymphocytes (white blood cells) start to grow uncontrollably. This is caused by a change, or mutation, in the DNA found in those cells. DNA mutations are common and don’t always result in cancer.

When a DNA mutation affects one or more critical genes, cancer, including B-cell lymphoma, can result. What causes this chain of events to occur is not readily known or understood.

B-cell lymphoma is not contagious and is not the result of anything you did or did not do.10 There are potential risk factors which may make you more vulnerable to certain types of B-cell lymphoma. They include:

Age
Being immunocompromised due to a condition such as HIV
Having an autoimmune condition such as rheumatoid arthritis, Sjögren’s syndrome, or lupus
Bacterial infections such as Helicobacter pylori (H. pylori)
Viral infections such as Epstein-Barr and hepatitis C

Getting a Diagnosis

Your healthcare provider will do tests and scans to determine if you have B-cell lymphoma. These tests will also help identify the type of lymphoma you have, its location, stage, and the extent of metastasis (spread). These factors are pivotal for creating an effective treatment plan.

Your doctor will start with a physical examination to look for swollen lymph nodes.

If swelling is found, a tissue biopsy of the lymph nodes will be done to look for abnormal cells. In this procedure, a sample is removed by a surgical procedure and analyzed in the lab. A biopsy may also be beneficial for determining the type of lymphoma you have, and how fast it is growing.11

Several blood tests may be done. They include12:

Complete blood count (CBC): Measures amounts of blood cells and lymphocytes
Blood smear: Identifies abnormal cells
Flow cytometry: Determines the types of proteins being manufactured by white blood cells
Serum immunoglobulin test: Measures antibody levels

If a diagnosis is made, additional tests may be done to determine metastasis (if and where the cancer has spread) and staging. They include:13

Bone marrow aspiration and biopsy: A sample is taken from the bone marrow of the hip or a long bone and examined in the lab.
Lumbar puncture: A sample of cerebrospinal fluid is taken from the spinal canal and analyzed in the lab.
Chest X-ray
Scans such as magnetic resonance imaging (MRI), computed tomography (CT) scan, positron-emission tomography (PET) scan, and bone scan
Abdominal sonogram (ultrasound)

Treatment for B-Cell Lymphoma

Your treatment will be determined by the type of B-cell lymphoma you have, as well as the stage of the disease. Your age and overall health will also be taken into account.

In some instances, treatment will be designed to cure the disease. In others, it will be geared toward reducing spread, controlling the disease, or alleviating symptoms.

Research into treatments for B-cell lymphoma is ever improving and ongoing. Currently, your doctor may recommend one or more of these treatments:14

Watchful waiting: If you have a slow-growing lymphoma, your doctor may recommend delaying treatment for a while. During this period, you'll have diagnostic checkups and tests that keep track of your symptoms and condition.
Chemotherapy: Chemotherapy uses medication to kill cancer cells throughout the body. It may be done alone or in conjunction with radiation. Chemotherapy drugs may be taken orally or administered via injection.
Central nervous system (CNS) prophylaxis: This is a type of chemotherapy in which medications are delivered into the spinal column via a lumbar puncture. It may be used to treat primary central nervous system lymphoma
Radiation: Radiation therapy may be done to kill cancer cells within the lymph nodes or to shrink tumors.
Targeted therapy: This treatment targets the proteins that control how cancer cells spread, grow, and divide.
Immunotherapy: This treatment uses laboratory-made substances called biologics to bolster the body’s immune system. Immunotherapy may also be used to kill or slow down the growth of lymphoma cells. 15
Stem cell transplants: Stem cell transplants replace diseased bone marrow with the patient’s own healthy stem cells (special cells that can develop into different types of cells). A donor’s stem cells may also be used. This treatment may be used along with radiation or chemotherapy

Prognosis

Your prognosis and five-year relative survival rate will be determined, in part, by the type of B-cell lymphoma you have and the stage of your cancer.

Keep in mind that the relative survival rate is an estimate, not a prediction about what will happen in your case. It’s also based on the experience of people who were diagnosed at least five years ago—before new treatments were being used.

The five-year relative survival rates for diffuse large B-cell lymphoma, based on patients diagnosed from 2010 to 2016, are:16

Localized (cancer that remains at the place it started): 73%
Regional (cancer that’s spread to nearby lymph nodes, tissues, or organs): 73%
Distant (cancer that has spread to distant areas of the body): 57%

The five-year relative survival rates for follicular lymphoma, based on patients diagnosed from 2010 to 2016, are:16

Localized: 96%
Regional: 90%
Distant: 85%

If you have aggressive lymphoma, your doctor may use the International Prognostic Index (IPI) to determine life-prolonging treatments and potential prognosis. This scale uses these five prognostic indicators:16

Age
Lymphoma stage
Metastasis into organs outside the lymphatic system
Your ability to participate in and complete daily life tasks (performance status)
Lactate dehydrogenase (LDH) blood levels, which can increase based on the amount of lymphoma you have

Some types of B-cell lymphoma, such as Burkitt lymphoma, diffuse large B-cell lymphoma, and marginal zone lymphoma, may be curable.

Other types, such as follicular lymphoma, are not usually curable but respond well to treatment. Even though they can’t be cured, many people live for years or even decades with these conditions.

Coping With a B-Cell Lymphoma Diagnosis

Getting a lymphoma diagnosis is never easy. If you have B-cell lymphoma, it can also be confusing since there are so many types and subtypes. A good place to start is with your doctor or medical team.

Ask as many questions as you need to, and don’t hesitate to request clarification when you’re confused or unsure. Accessing information can help you create a roadmap that will make coping with your diagnosis easier.

If you’re living with a chronic disease or are in the watchful waiting stage, it may be easy to procrastinate and avoid appointments. It’s very important that you adhere to your treatment plan and see your doctor for testing regularly.

The unknown is often scarier than the known. Staying on top of your treatment will help your prognosis, plus provide clarity that will enable you to better cope.

Maintaining healthy habits can also help you cope. Reducing stress, eating healthy food, and getting enough sleep can bolster your strength plus give you a sense of control. Try stress busters like yoga, meditation, and exercise. These strategies may also help you sleep better.

Many people benefit from having a support network. If you already have a caring network of family or friends in place, make sure to stay in touch with the people who can be there for you with a kind word or good advice long term.

You may consider joining a support group of people who are going through the same experience you are. One-on-one support from a therapist may also help. You can find groups as well as therapists through the Leukemia and Lymphoma Society.

Summary

B-cell lymphoma is a type of non-Hodgkin's lymphoma (NHL) that starts in a white blood cell that makes antibodies. The cancerous cells may be found in the lymph nodes, organs, or bone marrow. There are several types of B-cell lymphoma, and some are aggressive, while others are grow slowly.

Symptoms can include enlarged lymph nodes and spleen, fever, night sweats, and unintended weight loss. Diagnostic tests include blood tests, biopsies, and imaging. Treatment depends on the type of B-cell lymphoma and may consist of watchful waiting, chemotherapy, targeted therapy, immunotherapy, radiation, or a stem cell transplant.

Sources

American Cancer Society. Types of B-cell lymphoma.

Lymphoma Research Foundation. Diffuse large B-cell lymphoma.

Sogunro OA, Steinhauer R, Lewis E. T cell/histiocyte rich B-cell lymphoma: A difficult diagnosis to make. Current Problems in Cancer: Case Reports. 2020;2:100041. doi:10.1016/j.cpccr.2020.100041

Lymphoma Research Foundation. Follicular lymphoma.

National Organization for Rare Disorders. Mantel cell lymphoma.

Lymphoma Research Foundation. Mantle cell lymphoma: treatment options.

Cancer Research UK. Burkitt lymphoma.

National Organization for Rare Disorders. Primary central nervous system lymphoma.

Cancer Research UK. Diffuse large B cell lymphoma.

Lymphoma Action. Causes and risk factors for lymphoma.

Lymphoma Action. Biopsy.

Lymphoma Action. Blood tests.

RadiologyInfo. Lymphoma.

American Cancer Society. Treating B-Cell non-Hodgkin lymphoma.

American Cancer Society. Immunotherapy for non-Hodgkin lymphoma.