High-Grade B-Cell Lymphoma
High-grade B-cell lymphoma is an aggressive type of blood cancer that grows quickly and requires prompt treatment, but modern therapies can put many people into remission.
Table of contents
- What is High-Grade B-Cell Lymphoma?
- Other Names
- Types and Gene Changes
- Symptoms
- Diagnosis
- Treatment Options
- Outlook and Prognosis
- Related Conditions
What is High-Grade B-Cell Lymphoma?
High-grade B-cell lymphoma (HGBCL) is an aggressive, fast-growing type of non-Hodgkin lymphoma (a cancer that starts in white blood cells called lymphocytes)[1]. It develops when abnormal B cells, a specific type of white blood cell that helps fight infection, multiply rapidly and form tumors in your body[2].
This type of lymphoma is characterized by specific changes in genes within the cancer cells. One change involves the MYC gene, and the other involves either the BCL2 gene or, less commonly, the BCL6 gene[1]. When parts of genes switch places within chromosomes (the structures that carry genetic information), it’s called a rearrangement. These rearrangements cause the lymphoma cells to grow quickly.
High-grade B-cell lymphoma shares many features with two other types of B-cell lymphomas: diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma. In fact, about 5 percent of DLBCLs and about 32 to 78 percent of Burkitt lymphomas have rearrangements of the MYC and BCL2/BCL6 genes and are classified as HGBCL[1]. However, research has shown that HGBCL differs in several important ways from the forms of DLBCL and Burkitt lymphoma that do not have these gene rearrangements.
In 2016, the World Health Organization designated HGBCL as its own category of B-cell non-Hodgkin lymphoma[1]. This recognition helps doctors better understand the disease and choose the most appropriate treatments.
double-hit lymphoma, triple-hit lymphoma, HGBCL
Other Names
High-grade B-cell lymphoma was previously known as double-hit lymphoma[1]. It may also be called triple-hit lymphoma. The term “hits” refers to the number of genes in the lymphoma cells that have been changed[3].
Types and Gene Changes
High-grade B-cell lymphoma is classified based on which genes have rearrangements:
Double-hit lymphoma has rearrangements in the MYC gene and in either the BCL2 gene or the BCL6 gene[3].
Triple-hit lymphoma has rearrangements in the MYC gene, BCL2 gene, and BCL6 gene[3].
Molecular tests allow doctors to check for gene rearrangements in chromosomes under a microscope. These tests are used to confirm a diagnosis of HGBCL[1].
Symptoms
High-grade B-cell lymphoma causes symptoms similar to those of diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma[2]. Because it is an aggressive, fast-growing lymphoma, symptoms tend to develop quite quickly[6].
Common symptoms include:
- Swollen lymph nodes in the neck, underarms, or groin
- Drenching night sweats
- Fatigue (feeling very tired)
- Abdominal (belly) pain, if the lymphoma develops in the stomach or intestines[2]
High-grade B-cell lymphoma can spread to the brain and spinal cord, which together are called the central nervous system (CNS). Compared with other types of B-cell lymphomas, HGBCL cells are more likely to spread to the CNS[9].
Diagnosis
Diagnosis of high-grade B-cell lymphoma often begins with a physical exam. Your doctor will check for swollen lymph nodes in your neck, underarms, and groin, and for an enlarged spleen or liver[13].
Other tests and procedures include:
- Blood tests to measure levels of certain substances and to check for viruses. Blood tests may show whether lymphoma cells are present and can measure levels of lactate dehydrogenase (LDH), which is often higher in people with lymphoma[13].
- Imaging tests such as MRI, CT, and positron emission tomography (PET) scans to show the location and extent of the lymphoma[13].
- Biopsy, which involves removing a sample of tissue for testing in a lab. Your doctor may suggest a lymph node biopsy or a biopsy of other tissue to look for cancer cells. In the lab, tests will show whether you have high-grade B-cell lymphoma[13].
- Bone marrow aspiration and biopsy to collect cells from the bone marrow (the soft matter inside bones where blood cells are made). The samples are tested to see if the lymphoma has spread to the bone marrow[13].
Molecular tests that check for gene rearrangements in chromosomes are essential to confirm a diagnosis of HGBCL[1].
Treatment Options
Since high-grade B-cell lymphoma is a fairly new classification of lymphoma, ongoing research is helping doctors learn more about the best ways to treat this disease[9]. Your healthcare team will suggest treatments based on your needs and work with you to develop a treatment plan. When suggesting treatments, they will consider the stage of lymphoma, whether you have symptoms, your age, your general health, and your preferences[3].
Chemotherapy
Chemotherapy uses drugs to destroy cancer cells. It is the main treatment for high-grade B-cell lymphoma and is often combined with a targeted therapy drug[3].
HGBCLs are generally treated with one of the following combination chemotherapy regimens:
- DA-EPOCH-R, which includes dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin plus rituximab. This is the most common drug combination used to treat high-grade B-cell lymphoma[3][9].
- R-Hyper-CVAD, which includes rituximab plus hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone, alternating with high-dose methotrexate and cytarabine[9].
- R-CODOX-M/R-IVAC, which includes rituximab plus cyclophosphamide, vincristine, doxorubicin, and methotrexate, alternating with rituximab plus ifosfamide, etoposide, and cytarabine[9].
If high-grade B-cell lymphoma comes back after being treated (called a relapse) or doesn’t respond to treatment (called refractory disease), you may be offered other chemotherapy combinations[3].
You will receive chemotherapy in cycles. First you take a drug for a few days, and then you don’t take any medicine for a few days to let your body recover[15].
CNS Prophylaxis
To reduce the risk of the lymphoma spreading to the central nervous system (brain and spinal cord), patients with HGBCL may receive CNS prophylaxis in addition to one of the combination chemotherapy regimens[9]. CNS prophylaxis is administered through a lumbar puncture, which allows the doctor to inject one or more chemotherapy drugs directly into the cerebrospinal fluid (the fluid that surrounds the brain and spinal cord). This is called intrathecal chemotherapy[3]. Methotrexate is commonly used in CNS prophylaxis[3].
Stem Cell Transplant
A stem cell transplant replaces stem cells when stem cells, the bone marrow, or both are damaged. You may be offered an autologous stem cell transplant (a transplant using your own stem cells) for high-grade B-cell lymphoma that comes back after treatment or doesn’t respond to treatment[3].
Your healthcare team may suggest stem cell transplant as a treatment option if you are generally healthy, the lymphoma responds to chemotherapy, the lymphoma hasn’t spread to the central nervous system, and your organs are working normally[3]. Some patients with HGBCL may also undergo an allogeneic stem cell transplant (a transplant using stem cells from a donor)[9].
A stem cell transplant is very risky and complex, and it must be done in a special transplant center or hospital[3].
Targeted Therapy
Targeted therapy uses drugs to target specific molecules (such as proteins) on cancer cells or inside them. These molecules help the cancer cells grow and survive. Rituximab is a type of targeted therapy drug that is commonly combined with chemotherapy for treating high-grade B-cell lymphoma[3].
Outlook and Prognosis
High-grade B-cell lymphoma is an aggressive lymphoma that grows quickly. There are treatments that can put the condition into remission, meaning you don’t have symptoms and tests don’t find signs of cancer. However, the condition often comes back (recurs)[2].
With modern treatment of patients with aggressive B-cell lymphoma, more than 70 percent of patients can be cured. Most relapses occur in the first 2 years after therapy[8].
Related Conditions
High-grade B-cell lymphoma is closely related to other types of B-cell lymphomas:
Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma. About 5 percent of DLBCLs have the same gene rearrangements as HGBCL[1].
Burkitt lymphoma is a rare, fast-growing lymphoma. About 32 to 78 percent of Burkitt lymphomas have the gene rearrangements characteristic of HGBCL[1].
