High-grade B-cell lymphoma Burkitt-like lymphoma is a rare and aggressive form of blood cancer that develops from abnormal B-cells in the lymphatic system. This fast-growing condition shares features with both Burkitt lymphoma and diffuse large B-cell lymphoma, making accurate diagnosis essential for proper treatment.
Understanding the Disease
High-grade B-cell lymphoma Burkitt-like lymphoma represents a type of cancer that starts in the lymphatic system, which is the network of organs, vessels, and tissues that helps your body fight infections. When you have this condition, certain white blood cells called B lymphocytes begin to grow out of control and form tumors. These are the same cells that normally help protect you from infections by producing antibodies. In this disease, however, something goes wrong with these cells, causing them to multiply rapidly and spread to different parts of the body.[1]
This type of lymphoma is considered “high-grade,” which means it grows very quickly and requires immediate medical attention. The term “Burkitt-like” indicates that while this condition shares many similarities with classic Burkitt lymphoma, it has some differences in how the cells look under a microscope and how the disease behaves. These differences are important because they affect how doctors approach treatment and what outcomes patients can expect.[8]
The disease can develop in various places throughout the body. It commonly affects the jaw, central nervous system, bowel, kidneys, ovaries, and other organs. Because it grows so rapidly, the lymphoma can spread from the lymphatic system to other parts of the body relatively quickly if not treated promptly. The cancer cells may accumulate in lymph nodes or other body organs, where they interfere with normal function.[2]
Epidemiology
Burkitt lymphoma and Burkitt-like lymphoma are relatively uncommon diseases. In the United Kingdom, approximately 260 people receive a diagnosis of Burkitt lymphoma each year, which represents about 2 out of every 100 cases of non-Hodgkin lymphoma. While Burkitt lymphoma is the most common type of non-Hodgkin lymphoma in children in the UK, adults can also develop the disease, though this is less frequent.[4]
The disease shows a clear pattern in who it affects. Males are much more likely to develop this condition than females, with the disease affecting three to four times more males. This gender difference is consistent across different types of Burkitt lymphoma, though researchers are still working to understand exactly why this pattern exists.[7]
There are different forms of this disease that occur in different parts of the world. The endemic form, which is most common in equatorial Africa, New Guinea, and parts of South America, has an incidence rate about 50 times higher in these regions compared to the United States. This form is most frequently seen in children, especially boys. The sporadic form, which occurs throughout the world including the United States and Western Europe, accounts for less than 1% of B-cell non-Hodgkin lymphomas in adults but represents 30% of all childhood lymphomas.[2]
Causes
The development of high-grade B-cell lymphoma Burkitt-like lymphoma involves complex changes at the genetic level. A hallmark of this disease is a translocation, which is a type of genetic rearrangement, involving a gene called MYC. This gene normally helps control cell growth, but when it becomes rearranged, it can drive cells to multiply uncontrollably. This genetic change is critical for the development of the disease and is one of the key features doctors look for when making a diagnosis.[2]
In the endemic form of Burkitt lymphoma, nearly all cases are associated with the Epstein-Barr virus (EBV), a common virus that often causes symptoms similar to a cold or can lead to glandular fever. However, it’s important to understand that while EBV is very common in the general population, only a tiny fraction of people who have this virus ever develop lymphoma. In the sporadic form seen in Western countries, only a small proportion of cases show evidence of EBV infection.[3]
The connection between EBV and Burkitt lymphoma is particularly strong in areas where malaria is endemic. Researchers believe that chronic malaria infection may somehow work together with EBV to increase the risk of lymphoma development. In people with weakened immune systems, EBV-driven B-cell growth can lead to the accumulation of genetic mutations, particularly the MYC translocation, which drives the uncontrolled growth of B-cells and ultimately results in lymphoma.[3]
High-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements (sometimes called double-hit lymphoma) involves rearrangements in two particular genes. One rearrangement involves the MYC gene, and the other involves the BCL2 gene or, less commonly, the BCL6 gene. These genetic changes cause the cells to behave more aggressively than they would with just a single genetic change.[8]
Risk Factors
Certain groups of people face a higher risk of developing high-grade B-cell lymphoma Burkitt-like lymphoma. One of the most significant risk factors is having a weakened immune system. People living with HIV/AIDS are at increased risk for developing the immunodeficiency-related variety of this disease. The virus weakens the body’s ability to control B-cell growth, making it easier for cancerous changes to occur.[2]
Individuals who have received an organ transplant also face elevated risk. This is because they must take medications that suppress their immune system to prevent their body from rejecting the transplanted organ. These immunosuppressive medications can reduce the body’s ability to detect and eliminate abnormal cells, including cancer cells. People with inherited immune deficiencies similarly have a higher chance of developing this type of lymphoma.[2]
Geographic location plays a role in risk, particularly for the endemic form of the disease. Living in areas where malaria is holoendemic, such as equatorial Africa, Brazil, and Papua New Guinea, increases the risk of developing endemic Burkitt lymphoma. The interaction between chronic malaria infection and EBV appears to contribute to this increased risk, though the exact mechanism is still being studied.[3]
Symptoms
The symptoms of high-grade B-cell lymphoma Burkitt-like lymphoma typically develop rapidly and can worsen within just a few days. Because this is such a fast-growing cancer, symptoms often appear suddenly and progress quickly, which is why early detection and prompt medical evaluation are critical for anyone experiencing these signs.[2]
One of the most common presentations involves swelling in the abdomen. Sporadic Burkitt lymphoma, the type most commonly seen in Western countries, often grows in the abdomen and bowel. People with this may experience pain in the belly or back, feelings of nausea or actual vomiting, diarrhea, and noticeable swelling of the abdomen as fluid collects. In some cases, there may be bleeding. These abdominal symptoms can be severe and may be mistaken for other digestive problems initially.[4]
Many people notice painless swellings in specific areas of the body. These swellings represent enlarged lymph nodes and can appear in the neck, armpit, or groin. What makes these particularly concerning is how quickly they can grow—sometimes becoming noticeably larger over just a few days. The endemic form, common in Africa, often causes growths in the jaw, facial bones, or eye area. It can also affect other organs such as the thyroid, kidneys, and ovaries.[1]
General symptoms known as “B symptoms” are important warning signs. These include drenching night sweats that may soak through clothing or bedding, high temperatures that come and go without an obvious cause, and significant weight loss—specifically losing more than one-tenth of your total body weight without trying. Other general symptoms can include profound fatigue, weakness, decreased appetite, and a general feeling of being unwell.[2]
When Burkitt lymphoma grows in the bone marrow, it can take up the space normally occupied by healthy blood cells. This leads to low blood cell counts, which cause their own set of symptoms. People may experience tiredness and shortness of breath from low red blood cell counts, or easy bleeding and bruising from low platelet counts. If the lymphoma develops in the brain and spinal cord, it can cause headaches, confusion, seizures, or other neurological symptoms.[4]
Prevention
Because the exact causes of high-grade B-cell lymphoma Burkitt-like lymphoma are not fully understood, there are no guaranteed methods to prevent the disease. However, understanding the risk factors can help guide certain protective measures. For people living in areas where malaria is endemic, taking appropriate measures to prevent malaria infection may help reduce risk, given the known association between chronic malaria and the endemic form of Burkitt lymphoma.[3]
For individuals with HIV, maintaining good control of the virus through appropriate antiretroviral therapy is important. While having HIV increases the risk of developing immunodeficiency-related Burkitt lymphoma, proper treatment that keeps the immune system functioning as well as possible may help reduce this risk. Regular medical care and monitoring are essential for people living with conditions that affect their immune system.[2]
People who have received organ transplants need to take immunosuppressive medications to prevent organ rejection, which cannot be avoided. However, working closely with their medical team to use the lowest effective doses of these medications, when possible, while still preventing rejection, is important. Regular medical follow-up allows doctors to monitor for any early signs of lymphoma or other complications.[2]
Being aware of the symptoms and seeking prompt medical attention when they occur is perhaps the most important action anyone can take. Because this disease grows so rapidly, early diagnosis and immediate treatment initiation can make a significant difference in outcomes. Anyone experiencing rapidly developing symptoms such as quickly enlarging lymph nodes, severe abdominal pain, or persistent B symptoms should contact their healthcare provider without delay.[2]
Pathophysiology
At the cellular level, high-grade B-cell lymphoma Burkitt-like lymphoma represents a profound disruption of normal B-cell development and function. Normally, B lymphocytes develop in the bone marrow and mature in the lymphatic system, where they play a crucial role in fighting infections by producing antibodies. In this disease, genetic changes cause these cells to lose their normal regulatory controls and begin multiplying without the usual limits.[1]
The key genetic change in Burkitt lymphoma involves the MYC gene. This gene produces a protein that normally helps regulate cell growth and division. When a translocation occurs—meaning part of one chromosome breaks off and attaches to another chromosome—the MYC gene can end up in the wrong location, next to genes that are very active in B cells. This causes the MYC gene to become overactive, driving cells to divide much more rapidly than normal. This is why Burkitt lymphoma is one of the fastest-growing malignancies in humans, with a doubling time of around 25 hours and a growth fraction close to 100%.[6]
In cases involving EBV, the virus contributes to the disease process in specific ways. The EBNA1 protein, an EBV latent protein, is expressed in endemic Burkitt lymphoma. Changes in viral genes lead to the expression of EBNA3 genes, and tumor cells derived from these cell lines become resistant to normal cell death processes (apoptosis). This resistance to natural cell death gives the cancer cells a survival advantage, allowing them to accumulate even as they acquire additional harmful mutations.[3]
In high-grade B-cell lymphoma with rearrangements of both MYC and BCL2 or BCL6 genes, the disease process becomes even more complex. The BCL2 gene normally helps regulate cell death, and when it becomes rearranged along with MYC, cells gain the ability both to multiply rapidly (from MYC) and to resist dying (from BCL2). This combination makes the cancer particularly aggressive and harder to treat than lymphomas with only one of these genetic changes.[8]
As the abnormal B cells accumulate, they form tumors in lymph nodes and can spread to other organs. When they infiltrate the bone marrow, they crowd out normal blood-producing cells, leading to decreased production of red blood cells, white blood cells, and platelets. This explains many of the symptoms patients experience, such as fatigue from anemia, increased infections from low white blood cell counts, and bleeding problems from low platelet counts. The rapid growth of tumors can also cause mechanical problems, such as compression of nearby structures or blockage of the intestines when the disease involves the abdomen.[4]
