Burkitt Lymphoma: Patient Guide

This page is for people with Burkitt lymphoma and their families. It explains what makes this cancer different, how it is treated, and what to ask at the first visit.

Burkitt lymphoma starts in B cells, a type of white blood cell. It is an aggressive lymphoma that needs treatment soon after it is found.

Because it is aggressive, the team treats it with intense, multidrug chemotherapy and usually moves quickly.

The disease is defined by one change. The MYC gene, on chromosome 8, gets moved and switched on. That move is called a translocation, a swap of material between chromosomes. MYC lands next to an antibody gene and stays on.

NCI describes Burkitt lymphoma as marked by a translocation that turns on the MYC gene on chromosome 8. A translocation is a swap of material between chromosomes.

One line on your report matters enormously. Some lymphomas carry a second translocation, in BCL2, alongside MYC. NCI says patients with both MYC and BCL2 translocations appear to have an extremely poor outcome despite aggressive treatment (median overall survival 5 months). So if BCL2 appears next to MYC, ask one question. Is this Burkitt lymphoma, or a high-grade lymphoma with two rearrangements? The answer changes everything.

Endemic. Usually from Africa. It strikes the facial bones or jaws of children. Most of these tumors contain Epstein-Barr virus, or EBV, genes.

Sporadic. Usually involves the gut, the ovaries, or the kidneys.

Immunodeficiency-associated. Seen in people whose immune systems are weakened.

This part of treatment is not optional. NCI says people with Burkitt lymphoma have a 20 to 30 percent lifetime risk of spread to the brain and spinal cord. Because of that, preventive intrathecal chemotherapy is required. Intrathecal means the drug goes into the fluid around the spinal cord. Ask your team how many spinal taps (lumbar punctures) to expect.

In children, the rate at diagnosis is lower. A Berlin-Frankfurt-Munster analysis looked at more than 2,300 children with non-Hodgkin lymphoma. Among the Burkitt cases, 8.8 percent had spread to the brain or spinal cord.

For childhood non-Hodgkin lymphoma, NCI names the most widely used staging system. It comes from St. Jude Children's Research Hospital and is called Murphy staging. Ask your child's team how to read the stage on the report.

NCI describes aggressive multidrug regimens given together with rituximab. Rituximab is an antibody that targets CD20, a protein on B cells.

In children and teens, the Inter-B-NHL Ritux 2010 trial added rituximab to chemotherapy for aggressive mature B-cell lymphoma. Event-free survival (time without the cancer growing or coming back) rose from 82 percent to 94 percent, according to NCI.

Your team matches the amount of treatment to risk. NCI notes that most current pediatric protocols descend from the FAB/LMB 96 study.

Ask how closely spaced the cycles will be and what happens if one is delayed.

A large, fast tumor breaks apart quickly under chemotherapy. The contents of all those cells flood the blood at once. Uric acid rises sharply. That can damage the kidneys.

This is why rasburicase, sold as Elitek, often starts before or with the first chemotherapy. NCI says it is approved to treat high uric acid in the blood, in adults and children. It is also approved to prevent tumor lysis syndrome. That use covers leukemia, lymphoma, and solid tumors, when treatment is expected to drive uric acid up.

In practice, expect heavy fluids by vein and frequent blood draws. The first cycle is usually given in the hospital, not the clinic. Ask how often labs will be checked in the first 72 hours.

For adults, NCI reports more than 60 percent of advanced-stage patients free of disease at 5 years.

For children, the broader figure is better. Between 2013 and 2019, the 5-year relative survival rate was 90 percent. That figure covers children and teens under 20 with non-Hodgkin lymphoma.

Ask your team about the goal of treatment for your situation.

During diagnosis and the first cycles, these need contact straight away, at any hour. If you cannot reach the team, or someone is very unwell, call 911 or your local emergency number.

• Fever, especially any temperature over 100.4 degrees Fahrenheit.
• Passing much less urine than usual, or none.
• Muscle cramps, twitching, tingling around the mouth, or an irregular heartbeat.
• A new or worsening headache, double vision, facial numbness, or weakness in an arm or leg.
• A lump or swelling that is visibly larger than it was yesterday.
• Severe abdominal pain, vomiting, or inability to pass stool.

• Does my report show MYC alone, or MYC plus BCL2?
• Was EBV testing done on the tumor tissue?
• Which regimen am I getting, and how many cycles?
• What intrathecal treatment is planned, and how many lumbar punctures?
• What is the plan to prevent tumor lysis syndrome, and will I be admitted for it?
• How soon does treatment start, and what happens if there is a delay?

• NCI PDQ — Aggressive B-Cell Non-Hodgkin Lymphoma Treatment (Health Professional Version)
• NCI PDQ — Childhood Non-Hodgkin Lymphoma Treatment (Health Professional Version)
• NCI — Rasburicase