Burkitt Lymphoma: Patient Guide

Burkitt lymphoma starts in B cells, a type of white blood cell. It grows faster than almost any other human cancer. NCI describes extremely high growth rates. A mass can double in size over days, not months.

That speed cuts both ways. It makes this an emergency on the day it is found. It is also why hard chemotherapy works so well here. Fast-dividing cells are the ones chemotherapy hits hardest.

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 lists the three translocations seen:

• t(8;14)(q24;q32), the most common form.
• t(2;8)(p11;q24).
• t(8;22)(q24;q11).

Other genes are often altered too. NCI names TCF3, ID3, CCND3, and TP53.

One line on your report matters enormously. Some lymphomas carry a second translocation, in BCL2, alongside MYC. That is a different and far more dangerous disease. NCI reports a very poor outcome even with hard treatment. Median overall survival was 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 carry Epstein-Barr virus, or EBV. In sub-Saharan Africa, the rate of EBV-driven Burkitt lymphoma runs tenfold to twentyfold higher than in the United States.

Sporadic. The form seen in North America and Europe. It usually involves the gut, the ovaries, or the kidneys. In high-income countries, about 60 percent of cases start in the abdomen. Another 15 to 20 percent start in the head and neck. Only about 15 percent of cases in Europe and the US show EBV in the tumor. In Africa, almost all endemic cases do.

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

Rates differ by group in the United States. NCI reports Burkitt lymphoma as more common in non-Hispanic White people, at 3.2 cases per million person-years, than in Hispanic White people, at 2 cases per million person-years.

This part of treatment is not optional. NCI puts the lifetime risk of spread to the brain and spinal cord at 20 to 30 percent. Because of that, intrathecal chemotherapy is required to prevent it.

Intrathecal means the drug goes into the fluid around the spinal cord. It is given that way for a reason. Most chemotherapy given by vein does not reach the brain and spinal fluid well. Expect spinal taps in every regimen.

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. So a child's report will not read like a solid tumor stage. That is why the numbers do not line up with what you may expect.

Burkitt lymphoma is not treated like slower lymphomas. NCI describes intense multidrug regimens given with rituximab. Rituximab is an antibody that targets CD20, a protein on B cells. Adult treatment here is modeled on regimens built for children.

Adding rituximab produced a clear jump. The Inter-B-NHL Ritux 2010 trial tested it in aggressive mature B-cell lymphoma. Event-free survival rose from 82 percent to 94 percent.

The amount of treatment is matched to risk. In NCI's table of standard pediatric options, completely resected stage I and abdominal stage II disease gets only two cycles of chemotherapy, while mature B-cell leukemia or CNS disease gets six cycles plus rituximab. Most current pediatric protocols descend from the FAB/LMB 96 study.

Cycles are given close together, with little gap. That is on purpose. A delay gives the tumor time to grow back.

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.

Here, cure is the goal from day one. It is not a long shot.

During diagnosis and the first cycles, these need same-day contact:

• 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?

https://www.cancer.gov/types/lymphoma/hp/aggressive-b-cell-lymphoma-treatment-pdq

https://www.cancer.gov/types/lymphoma/hp/child-nhl-treatment-pdq

https://www.cancer.gov/about-cancer/treatment/drugs/rasburicase