Burkitt’s Lymphoma

Burkitt’s lymphoma is a rare but fast-growing cancer that develops in the body’s infection-fighting system. Though it can be life-threatening because of how quickly it spreads, most people respond well to treatment and can go into long-term remission when diagnosed and treated right away.

Table of contents

• What Is Burkitt’s Lymphoma?
• Types of Burkitt’s Lymphoma
• Symptoms and Warning Signs
• What Causes Burkitt’s Lymphoma
• How Doctors Diagnose This Condition
• Disease Stages
• Treatment Options
• What to Expect: Prognosis and Outlook
• Possible Complications

What Is Burkitt’s Lymphoma?

Burkitt’s lymphoma is a type of cancer that affects the lymphatic system, which is the network of organs, glands, tubes, and clusters of cells called lymph nodes that help your body fight infections and disease^[1]. It belongs to a larger group of cancers called non-Hodgkin lymphoma^[1].

This cancer starts in a type of white blood cell called B cells or B lymphocytes, which normally help your immune system protect you from germs and illness^[2]. In Burkitt’s lymphoma, changes happen that turn these B cells into cancer cells. These abnormal cells can build up in lymph nodes or other parts of your body where they don’t work properly and can’t fight infection as normal white blood cells do^[4].

Burkitt’s lymphoma is known as a highly aggressive or fast-growing cancer^[3]. The tumors can double in size within just a few days^[2]. The cancer cells can grow in many places including the lymph nodes, jaw, other facial bones, parts of the intestines, bone marrow, central nervous system, and other areas of the body^[1].

While Burkitt’s lymphoma is rare, affecting about 4 in 1 million children under 16 in the United States, it makes up about 2% of all non-Hodgkin lymphoma cases^[2][4]. Each year around 260 people are diagnosed with this condition in the United Kingdom^[4]. Burkitt’s lymphoma is more common in males than females^[4].

Burkitt’s disease, Burkitt’s tumor, Burkitt lymphoma, malignant lymphoma Burkitt’s type

Types of Burkitt’s Lymphoma

There are three main types of Burkitt’s lymphoma, classified based on where they occur and what causes them^[1]:

Endemic Burkitt’s lymphoma is most commonly found in Africa, particularly in equatorial Africa, New Guinea, and parts of South America and Brazil^[1][3]. It has an incidence rate about 50 times higher in equatorial Africa and New Guinea than in the United States^[3]. This type is the most common childhood cancer overall in these regions^[3]. It more commonly affects children, especially boys^[1]. Nearly everyone with endemic Burkitt’s lymphoma also has Epstein-Barr virus (EBV), a common virus that often causes symptoms similar to a cold^[1]. The most common site of disease is the jaw, though it can also affect the thyroid, kidneys, ovaries, and other facial bones or the eye area^[1][3]. Some research suggests a link with malaria and EBV^[4].

Sporadic Burkitt’s lymphoma occurs throughout the world, particularly in the United States and Western Europe^[3][4]. This is the most common type of Burkitt’s lymphoma seen in the United Kingdom and United States^[4]. It accounts for less than one percent of B-cell non-Hodgkin lymphomas in adults. However, it makes up about 30 to 40 percent of all childhood lymphomas^[3][2]. The sporadic type is sometimes linked to Epstein-Barr virus, though only about 20-30% of cases show evidence of EBV infection^[4][6]. An abdominal tumor is the most common site where disease occurs^[3]. Children are more likely to get it, usually between the ages of 3 and 12^[2].

Immunodeficiency-related Burkitt’s lymphoma affects people with weakened immune systems^[2]. This type is most common in people with human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS)^[3]. It accounts for 30% to 40% of non-Hodgkin lymphoma in HIV patients^[8]. It can also occur in patients who have inherited immune deficiencies or those who take immunosuppressive medications to prevent rejection after an organ transplant^[3][4].

Symptoms and Warning Signs

Burkitt’s lymphoma symptoms can appear suddenly and progress quickly. They can start or get worse in just a few days^[4][3]. Common symptoms include^[2][3][4]:

• Belly pain, nausea, or vomiting
• Fever, tiredness, and night sweats
• Loss of appetite and unintended weight loss
• Swollen lymph nodes in the neck, armpit, or groin
• Rapidly growing tumor or mass, often in the abdomen

The symptoms depend on where the lymphoma grows in your body. Sporadic Burkitt’s lymphoma often grows in the abdomen and bowel. When this happens, you might experience tummy or back pain, feeling or being sick, diarrhea, swelling of your abdomen as fluid collects, or bleeding^[4].

You might have one or more painless swellings in the neck, armpit, or groin. These are enlarged lymph nodes that can grow very quickly^[4].

Some people experience what doctors call B symptoms, which include heavy sweating at night, high temperatures that come and go with no obvious cause, and losing a lot of weight (more than one tenth of your total weight)^[4].

If Burkitt’s lymphoma grows in the bone marrow, lymphoma cells can take up the space of normal blood cells. This can cause low blood cell counts, with symptoms including tiredness, shortness of breath, and bleeding and bruising^[4].

Less than 10% of cases present with central nervous system involvement, which usually appears as cranial nerve problems or spinal cord compression^[6].

These symptoms can look like common illnesses, but if they get worse, you should see a healthcare provider. Because Burkitt tumors can double in size within days, early detection and prompt medical evaluation are critical^[2][3].

What Causes Burkitt’s Lymphoma

Experts don’t know exactly what causes Burkitt’s lymphoma. The disease is linked to changes in a gene called MYC, which helps control cell growth^[2][3]. A type of genetic change called translocation (a type of rearrangement) can cause this gene to become overactive^[2][3]. This translocation of the MYC gene is a hallmark of Burkitt’s lymphoma, making it an important finding for diagnosis^[3].

Epstein-Barr virus (EBV) infection is a known risk factor. This is the virus that causes glandular fever (mononucleosis)^[4]. While nearly all endemic cases are associated with EBV infection, only a small proportion of sporadic cases show evidence of EBV infection^[5]. In the United States, some people who have Epstein-Barr virus also have Burkitt’s lymphoma, but it’s important to remember that EBV is very common and not everyone who has EBV develops Burkitt’s lymphoma^[2][4].

In immune-compromised individuals, EBV-driven B-cell growth leads to the accumulation of genetic mutations, particularly the MYC translocation. This drives the uncontrolled growth of B-cells, ultimately resulting in the development of Burkitt’s lymphoma^[5].

Burkitt’s lymphoma is also found in areas where malaria is always present, such as Brazil, Papua New Guinea, and equatorial Africa^[5]. Chronic malaria is believed to reduce resistance to EBV, allowing EBV infection to occur^[6].

How Doctors Diagnose This Condition

Burkitt’s lymphoma diagnosis often begins with a physical exam that checks for swollen lymph nodes and a neurological exam, which tests how well the brain and nerves are working^[9].

Blood tests can sometimes show whether lymphoma cells are present. They also measure levels of lactate dehydrogenase (LDH), an enzyme that is often higher in people with lymphoma. Blood tests can also check for infections that may increase the risk of Burkitt’s lymphoma, such as Epstein-Barr virus and HIV^[9].

Imaging tests make pictures of the body and can show the location and extent of Burkitt’s lymphoma. Tests might include CT scans (which look for tumors in your chest, belly, or pelvis) and PET scans (which highlight areas with cancer)^[2][9]. Your healthcare team may also do an MRI if they think the cancer is affecting the central nervous system^[9].

A lymph node biopsy is a procedure to remove a sample of tissue for testing in a lab. Your healthcare provider may suggest this to look for cancer cells. It involves removing all or part of a lymph node. In the lab, tests may show whether you have Burkitt’s lymphoma^[9].

Bone marrow aspiration and biopsy are procedures to collect cells from the bone marrow for testing. Bone marrow is the soft matter inside bones where blood cells are made. In a bone marrow aspiration, a needle is used to draw a sample of the fluid. In a bone marrow biopsy, a needle is used to collect a small amount of the solid tissue. The samples are typically taken from the hip bone and checked to see if cancer is in the marrow^[2][9].

A spinal tap (lumbar puncture) looks for cancer cells in spinal fluid^[2].

In adults, Burkitt’s lymphoma is often hard to tell apart from diffuse large B-cell lymphoma (DLBCL), another aggressive B-cell lymphoma^[3]. Accurate diagnosis of Burkitt’s lymphoma is critical because Burkitt’s lymphoma and DLBCL are treated differently. That is why it is strongly recommended that patients seek an opinion from an expert in lymphoma^[3].

Disease Stages

Healthcare providers use stages to show how far the cancer has spread^[2]:

• Stage I (1): One area or lymph node is affected
• Stage II (2): Cancer is in two or more areas on the same side of your breathing muscle (diaphragm)
• Stage III (3): Cancer is on both sides of your diaphragm
• Stage IV (4): Cancer has spread outside your lymph system (like to your liver, lungs, or bone marrow)

The bone marrow is involved in 30-35% of cases and the central nervous system is involved in approximately 20% of cases (usually affecting the covering of the brain and spinal cord)^[6].

Treatment Options

Because Burkitt’s lymphoma grows fast, treatment needs to start quickly. Burkitt’s lymphoma is a very fast-growing tumor, and patients should be admitted to the hospital for rapid workup and treatment^[12]. Treatment often includes a combination of approaches^[1][2]:

Chemotherapy is the main treatment and mainstay of care for Burkitt’s lymphoma^[2][12]. It is often intense and started right away. Since Burkitt’s lymphoma is very aggressive, the diagnosis of this disease requires urgent hospitalization and the immediate start of therapy^[10]. However, Burkitt’s lymphoma is often very responsive to the currently recommended intensive chemotherapy regimens, and cure rates for this disease remain high^[10].

Specific chemotherapy treatment options for adults include different combination regimens. All of the treatments used are intensive, using high doses of drugs given frequently^[10]. Different combination chemotherapy regimens are used to treat Burkitt’s lymphoma in children and adolescents. Younger patients tend to have excellent responses to chemotherapy and particularly high cure rates^[10].

Immunotherapy helps your immune system find and attack cancer^[2]. Treatment usually includes chemotherapy together with a targeted drug called rituximab. Doctors call this chemoimmunotherapy^[4].

Radiation therapy is sometimes used with chemotherapy, mostly for adults^[2]. However, no role exists for radiation therapy in most cases of Burkitt’s lymphoma treatment^[12].

Stem cell transplant (also called bone marrow transplant) may help if cancer returns after treatment^[1][2].

Surgery is used in rare cases, like when a tumor blocks your intestines^[2]. Generally, no role exists for surgery in the treatment of Burkitt’s lymphoma^[12].

If feasible, having the patient enroll in a clinical trial is strongly encouraged, because a preferred chemotherapy regimen for Burkitt’s lymphoma has yet to be established^[12].

Monitoring and preventive measures for tumor lysis syndrome and kidney problems are essential, especially during chemotherapy. Patients should receive preventive medication and aggressive hydration before starting chemotherapy^[12]. Most patients with Burkitt’s lymphoma are considered at high risk for tumor lysis syndrome^[12].

What to Expect: Prognosis and Outlook

Because it grows so fast, Burkitt’s lymphoma can be life-threatening. But with quick treatment, many people go into long-term remission. That means symptoms go away and stay away^[2]. Healthcare providers often treat Burkitt’s lymphoma with intensive chemotherapy, and most of the time, this cancer goes into long-term remission after treatment^[2].

The disease prognosis is excellent in children with intense chemotherapy treatment^[5]. With intensive chemotherapy, the overall cure rate for Burkitt’s lymphoma in developed countries is about 90%^[6]. However, intensive chemotherapy can achieve long-term survival in more than half the people with Burkitt’s lymphoma^[8].

Burkitt’s lymphoma is uncommon in adults, in whom it has a worse prognosis^[6]. The disease prognosis is poor in adults compared to children^[5].

Patients in remission from Burkitt’s lymphoma should have regular visits with a physician who is familiar with their medical history and the treatments they have received. Medical tests (such as blood tests and CT scans) may be required at various times during remission to evaluate the need for additional treatment^[19].

Possible Complications

Possible complications of Burkitt’s lymphoma include^[2]:

• Low blood cell counts, which may require transfusion of blood products
• Tumor lysis syndrome – a condition where cancer cells break down rapidly during treatment, releasing substances into the blood that can be harmful
• Weakened immunity

You should tell your healthcare provider if your symptoms become hard to manage^[2].

• Lymphatic system
• Lymph nodes
• Jaw
• Facial bones
• Abdomen
• Intestines
• Bone marrow
• Central nervous system
• Kidneys
• Ovaries
• Spleen
• Throat
• Tonsils