Burkitt lymphoma is a rare and extremely fast-growing cancer that affects a specific type of white blood cell responsible for fighting infections. Although it is aggressive in nature, early detection and prompt treatment with intensive therapy can lead to long-term remission for many patients, particularly children.
Understanding Burkitt Lymphoma
Burkitt lymphoma is a type of cancer that begins in the body’s lymphatic system, which is a network of organs, vessels, and small structures called lymph nodes that work together as part of the immune system to protect against disease and infection. This particular cancer affects B cells, also known as B lymphocytes, which are white blood cells that normally help the body fight off harmful germs and illnesses. When Burkitt lymphoma develops, changes occur in these B lymphocytes that turn them into cancer cells that grow and multiply very rapidly.[1]
The disease is classified as a form of non-Hodgkin lymphoma, one of two main categories of lymphoma, the other being Hodgkin lymphoma. What sets Burkitt lymphoma apart from many other cancers is its extraordinary speed of growth. The cancer cells can multiply so quickly that tumors may double in size within just a few days, making this one of the fastest-growing human tumors known to medicine.[2][8]
Burkitt lymphoma can develop in various parts of the body. The cancer cells may grow in lymph nodes, the jawbone or other facial bones, parts of the intestines and abdomen, bone marrow, the central nervous system, and other organs including the kidneys, ovaries, thyroid, spleen, throat, and tonsils.[1][2]
Epidemiology: How Common Is This Cancer?
Burkitt lymphoma is considered a rare cancer, though its frequency varies significantly depending on where in the world you live. 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.[4] In the United States, around 1,200 people are diagnosed annually, and the disease makes up only about 1% to 2% of adult lymphomas.[2][8]
One of the most striking patterns about this disease is how it affects different age groups. Burkitt lymphoma is much more common in children than in adults. In fact, it represents the most common type of non-Hodgkin lymphoma in children living in the United Kingdom. Among children diagnosed with non-Hodgkin lymphoma, Burkitt lymphoma accounts for approximately 30% to 40% of all cases.[4][16] In the United States, sporadic Burkitt lymphoma affects about 4 in every 1 million children under the age of 16.[2]
Children typically develop this cancer between the ages of 3 and 12 years old.[2] While adults can certainly be affected, diagnosis is much less common in older age groups. Among adults who do develop the disease, about 59% of patients are over the age of 40 at the time of diagnosis.[8]
There is also a clear difference in how the disease affects males versus females. Burkitt lymphoma is considerably more common in males, occurring three to four times more frequently in boys and men than in girls and women.[4][6]
The geographic distribution of Burkitt lymphoma is particularly noteworthy. The endemic form of the disease, which is most strongly linked to infection with the Epstein-Barr virus, is far more common in equatorial Africa and Papua New Guinea. In these regions, the incidence rate is approximately 50 times higher than in the United States.[3][16] In Africa, endemic Burkitt lymphoma is not just the most common type of Burkitt lymphoma but also the most common childhood cancer overall in affected areas.[16]
Types of Burkitt Lymphoma
Medical experts recognize three distinct types of Burkitt lymphoma based on where they occur and what factors are associated with them. Understanding these types helps doctors determine the best approach to treatment and what to expect during the course of the disease.
The endemic type is most commonly found in Africa, particularly in equatorial regions, as well as in parts of Papua New Guinea and South America. This form primarily affects children, especially boys, and has a very strong connection to the Epstein-Barr virus, or EBV. Nearly everyone diagnosed with endemic Burkitt lymphoma has evidence of EBV infection.[1][2] The most common location for tumors in this type is the jaw and facial bones, though the disease can also affect the thyroid, kidneys, ovaries, and eye area. Research suggests that chronic malaria infection may reduce the body’s resistance to EBV, allowing the virus to more easily trigger the changes that lead to cancer.[5][6]
The sporadic type occurs throughout the world, including in the United States and Western Europe. This is the most common form of Burkitt lymphoma found in the UK and represents the type most children and adults in developed countries will encounter. Unlike the endemic form, only about 20% to 30% of sporadic cases are associated with EBV infection.[6] In sporadic Burkitt lymphoma, the abdomen is the most common site where the disease develops, often affecting the bowel and right lower section of the abdomen. The jaw is less frequently involved compared to the endemic type.[4][6]
The immunodeficiency-related type develops in people whose immune systems are not functioning properly. This includes individuals living with HIV/AIDS, people who have received organ transplants and must take medications to suppress their immune system, and those with inherited immune deficiencies.[1][2][4] Before highly effective antiretroviral therapy became available for HIV, the incidence of Burkitt lymphoma in HIV-positive individuals was estimated to be 1,000 times higher than in the general population.[8] This form accounts for 30% to 40% of non-Hodgkin lymphoma cases in people with HIV.[8]
Causes and Origins
Scientists do not know the exact cause of Burkitt lymphoma, but they have identified several important factors that contribute to its development. The disease is strongly associated with specific genetic changes and viral infections that work together to transform normal B cells into cancer cells.
A key feature of Burkitt lymphoma is a genetic abnormality involving a gene called MYC. This gene normally helps control how cells grow and divide. In Burkitt lymphoma, a type of genetic mistake called a translocation occurs. A translocation happens when a piece of one chromosome breaks off and attaches to a different chromosome. When this involves the MYC gene, it becomes overactive and drives cells to grow and multiply uncontrollably.[2][3][5] This MYC translocation is considered a hallmark of Burkitt lymphoma and is an important finding doctors look for when making a diagnosis.[3]
The Epstein-Barr virus plays a significant role in the development of Burkitt lymphoma, though the relationship varies by type. EBV is the same common virus that causes infectious mononucleosis, often called glandular fever or “mono.” Most people are infected with EBV at some point in their lives, but only a tiny fraction ever develop lymphoma.[4] The virus is found in nearly all cases of endemic Burkitt lymphoma in Africa, but in only about 20% to 30% of sporadic cases and 30% to 40% of immunodeficiency-related cases.[6][8]
In people with weakened immune systems, particularly those with HIV, EBV-driven B-cell proliferation can lead to the accumulation of genetic mutations, especially the MYC translocation. This drives uncontrolled growth of B cells and ultimately results in the development of Burkitt lymphoma.[5] The weakened immune system is less able to keep EBV-infected cells under control, allowing them to multiply and acquire additional harmful mutations.
Chronic malaria infection appears to be another contributing factor, particularly in regions where the endemic form is common. Malaria is believed to reduce the body’s resistance to EBV, making it easier for the virus to cause the cellular changes that lead to cancer.[5][6] This helps explain why Burkitt lymphoma is so much more common in areas of Africa, Brazil, and Papua New Guinea where malaria is constantly present.
Risk Factors
Certain groups of people and particular circumstances increase the likelihood of developing Burkitt lymphoma. Understanding these risk factors can help identify individuals who may benefit from closer medical monitoring.
One of the most significant risk factors is having a weakened immune system. People living with HIV or AIDS face a substantially higher risk of developing Burkitt lymphoma. Before modern HIV treatments became available, individuals with HIV were 1,000 times more likely to develop this cancer than those without the virus.[8] Even with current treatments, HIV-positive individuals remain at elevated risk, and Burkitt lymphoma may sometimes be the first sign that someone has AIDS.
Individuals who have received organ transplants and must take immunosuppressive medications to prevent rejection of the transplanted organ also face increased risk.[3][4] These medications intentionally weaken the immune system to protect the new organ, but this also reduces the body’s ability to control abnormal cell growth and fight off viruses like EBV.
People born with inherited immune deficiencies that affect how their immune system functions are another at-risk group.[3][16] These genetic conditions mean the immune system never works at full strength, making it harder to control infections and prevent abnormal cells from becoming cancerous.
Living in regions where malaria is constantly present significantly increases risk, particularly for children. In equatorial Africa, Papua New Guinea, and parts of Brazil where malaria is endemic, the combination of chronic malaria infection and exposure to EBV creates conditions that make Burkitt lymphoma far more common.[5][6]
Being male is also associated with higher risk. Boys and men are three to four times more likely to develop Burkitt lymphoma than girls and women, though the reasons for this difference are not fully understood.[4][6]
Age plays an important role as well. Children, particularly those between ages 3 and 12, are more commonly affected than adults.[2] However, adults over age 40 who do develop the disease often face a more challenging prognosis.
Symptoms and How They Affect Patients
The symptoms of Burkitt lymphoma can appear suddenly and deteriorate rapidly, sometimes worsening noticeably within just a few days. This rapid progression is directly related to how quickly the cancer cells multiply and spread. The specific symptoms a person experiences often depend on where in the body the lymphoma is growing.
One of the most common symptoms is swelling in the lymph nodes. These swellings typically appear in the neck, armpit, or groin area and are usually painless. Unlike swollen lymph nodes caused by common infections, these lymph nodes can grow very quickly and become quite large.[3][4][16] The rapid growth can be alarming and is often what prompts people to seek medical attention.
When Burkitt lymphoma develops in the abdomen, which is particularly common in the sporadic type, patients may experience severe belly pain or back pain. The abdomen may become swollen and distended as fluid collects or as tumors grow. People often feel nauseated and may vomit. Changes in bowel habits, including diarrhea, are common. Some individuals experience bleeding from the intestines.[3][4][16] These symptoms can become severe enough to require emergency medical care.
General symptoms that affect the whole body are also common and are sometimes referred to as “B symptoms” by doctors. These include fever that comes and goes with no obvious cause, heavy night sweats that may soak through clothing and bedding, and significant unintended weight loss, specifically losing more than one-tenth of total body weight.[3][4][16] These symptoms occur because the cancer affects how the body functions overall.
Patients commonly experience extreme tiredness and weakness that doesn’t improve with rest. Loss of appetite is frequent, making it difficult to eat enough to maintain weight and strength.[2][3][16]
In endemic Burkitt lymphoma, particularly in African children, the jaw and facial bones are commonly affected. This causes visible swelling and distortion of the face, disruption of teeth alignment, and sometimes difficulty breathing if the airway becomes partially blocked.[1][6]
When the cancer spreads to the bone marrow, it can interfere with normal blood cell production. This leads to tiredness and shortness of breath from low red blood cell counts, and easy bleeding and bruising from low platelet counts.[4][14]
If Burkitt lymphoma affects the central nervous system, which occurs in about 20% to 35% of cases, symptoms may include headaches, changes in sensation or movement, problems with coordination, or cranial nerve palsies that affect functions controlled by nerves in the head.[6]
Prevention Strategies
Because the exact causes of Burkitt lymphoma are not fully understood, and because it involves genetic changes that occur spontaneously, there are no guaranteed ways to prevent the disease. However, certain measures may help reduce risk, particularly for people in high-risk groups.
For individuals with HIV, maintaining effective treatment with antiretroviral therapy is crucial. Keeping the immune system as strong as possible through consistent medication adherence significantly reduces the risk of developing lymphomas, including Burkitt lymphoma.[8] Regular medical monitoring and prompt treatment of infections also help maintain immune function.
In areas where malaria is endemic, preventing and treating malaria infections may help reduce the risk of endemic Burkitt lymphoma. This includes using mosquito nets treated with insecticide, taking antimalarial medications when recommended, and seeking prompt treatment for malaria symptoms.[5][6] By reducing the burden of chronic malaria, the immune system may be better able to control EBV infections.
For people who have received organ transplants, working closely with transplant specialists to find the right balance of immunosuppressive medications is important. While these medications are necessary to prevent organ rejection, doctors aim to use the lowest effective doses to minimize the risk of complications like lymphoma.
Early recognition of symptoms is perhaps the most important protective measure. Because Burkitt lymphoma grows so rapidly, recognizing warning signs and seeking immediate medical attention can lead to earlier diagnosis when treatment is most likely to be successful. Parents of children in high-risk groups should be particularly vigilant about unexplained swellings, persistent fever, or rapid changes in health.
How the Disease Changes Body Function
Understanding what happens inside the body when Burkitt lymphoma develops helps explain both the symptoms patients experience and why the disease requires such aggressive treatment. The changes begin at the cellular level but eventually affect multiple body systems.
Burkitt lymphoma starts when normal B lymphocytes undergo genetic changes, particularly the translocation involving the MYC gene. This translocation causes the gene to become overactive, driving the B cells to grow and divide at an extraordinary rate. These rapidly multiplying cells no longer function as normal immune cells should. Instead of helping fight infections, they crowd out healthy cells and form tumors in various parts of the body.[2][5]
When examined under a microscope, Burkitt lymphoma cells have a characteristic appearance. They divide so rapidly that when pathologists look at tissue samples, they often see a “starry sky” pattern. This pattern comes from the presence of normal white blood cells called macrophages that are cleaning up debris from the rapidly dying cancer cells, appearing as lighter spots against a dark background of densely packed lymphoma cells.[6]
As the cancer cells accumulate in lymph nodes, they cause the nodes to swell rapidly. The speed of growth distinguishes this from the slower enlargement seen in many other conditions. In the abdomen, tumors can grow large enough to cause obstruction of the intestines, compress other organs, or cause fluid to accumulate, leading to the visible swelling and severe pain many patients experience.[3]
When Burkitt lymphoma cells invade the bone marrow, they physically take up space that should be occupied by normal blood-forming cells. This process, called bone marrow infiltration, disrupts the production of red blood cells, white blood cells, and platelets. The reduction in red blood cells causes anemia, leading to fatigue and shortness of breath because tissues don’t receive enough oxygen. Low platelet counts cause easy bleeding and bruising because blood cannot clot properly. Reduced production of normal white blood cells weakens the immune system further, making patients more vulnerable to infections.[4][14]
One of the most serious complications of Burkitt lymphoma is called tumor lysis syndrome. This occurs when cancer cells die rapidly, either spontaneously or at the start of chemotherapy treatment. When millions of cells break down simultaneously, they release their contents into the bloodstream all at once. This floods the blood with substances like potassium, phosphate, and uric acid, which can overwhelm the kidneys’ ability to filter and eliminate them. The result can be dangerous changes in blood chemistry, kidney damage, and potentially life-threatening complications affecting the heart and other organs.[2][12]
When the central nervous system becomes involved, lymphoma cells can grow in the membranes covering the brain and spinal cord or within the brain tissue itself. This can increase pressure inside the skull, compress nerves, and interfere with normal brain function. The blood-brain barrier, which normally protects the brain from harmful substances, also makes it harder for some medications to reach cancer cells in this area, requiring special treatment approaches.[6]
The rapid growth of Burkitt lymphoma tumors also means they consume large amounts of nutrients and energy that the body would normally use for other functions. This contributes to the weight loss, fatigue, and overall decline in physical condition that patients experience. The body’s metabolism is disrupted as it tries to cope with the abnormal demands of rapidly multiplying cancer cells.
Throughout all of this, the normal immune system is severely compromised. Not only are the cancer cells non-functional as immune cells, but they also crowd out healthy lymphocytes and other immune cells. Additionally, the disease often develops in people whose immune systems are already weakened by HIV, immunosuppressive medications, or other conditions. This double burden means patients are highly vulnerable to infections that their bodies would normally fight off easily.[2]
