Burkitt lymphoma is a rare and aggressive form of non-Hodgkin lymphoma that affects a type of white blood cell called B-lymphocytes. It is one of the fastest-growing human cancers, with tumors capable of doubling in size in under a day. Despite its aggressive nature, Burkitt lymphoma is often highly responsive to treatment, and many individuals can be cured.
Clinical Presentation and Symptoms
The signs of Burkitt lymphoma often develop quickly, over a matter of days or weeks, and can vary depending on where the tumor is located. A common initial symptom is the rapid and noticeable swelling of lymph nodes, which can appear in the neck, armpits, or groin. The abdomen is another frequent site, where tumors can cause significant swelling, pain, nausea, vomiting, and changes in bowel habits.
In some cases, the disease first manifests in the bones of the face or jaw, leading to distortion and swelling. This presentation is particularly characteristic of the type of Burkitt lymphoma found in specific regions of Africa. Beyond localized tumors, individuals may also experience a set of systemic signs referred to as “B symptoms.” These include fevers with no obvious cause, drenching night sweats that can soak through clothing, and significant, unexplained weight loss.
The rapid growth of tumors can also lead to more acute complications. For example, a large abdominal mass might cause a blockage in the intestines or even lead to perforation. If the cancer spreads to the central nervous system (CNS), which includes the brain and spinal cord, it can cause headaches, confusion, or weakness.
Subtypes and Associated Risk Factors
Medical professionals recognize three distinct clinical subtypes of Burkitt lymphoma, each associated with different geographic patterns and risk factors. The endemic form is primarily found in equatorial Africa and is strongly linked to chronic malaria infection. The sporadic subtype occurs worldwide, including in North America and Europe. The third type, immunodeficiency-related Burkitt lymphoma, is most often seen in individuals with compromised immune systems, such as those with HIV/AIDS or patients who have received organ transplants.
A significant factor in the development of this cancer is the Epstein-Barr virus (EBV), the same virus that causes infectious mononucleosis. EBV is found in nearly all cases of endemic Burkitt lymphoma and a substantial portion of immunodeficiency-related cases. The virus is thought to play a part in the transformation of B-cells into cancerous cells, particularly when the immune system is suppressed by another condition like malaria or HIV.
At the molecular level, all forms of Burkitt lymphoma share a defining genetic characteristic: a translocation involving the c-MYC gene on chromosome 8. A translocation is a genetic mistake where a piece of one chromosome breaks off and attaches to another. In this case, the c-MYC gene is moved to a new location where it becomes constantly active, forcing the B-cells to divide uncontrollably.
The Diagnostic Process
Confirming a diagnosis of Burkitt lymphoma requires a multi-step process that begins with a tissue biopsy. A surgeon removes a piece of an enlarged lymph node or other suspicious tumor, which is then sent to a pathologist for examination under a microscope. Pathologists look for a specific appearance of the cancer cells, which are uniform in size and create a pattern often described as a “starry sky.”
Once the diagnosis is confirmed, the next step is staging, which determines how far the cancer has spread. This process is necessary for planning treatment and involves a series of tests:
- Computed tomography (CT) scans and positron emission tomography (PET) scans are used to create detailed pictures of the inside of the body, highlighting areas where the cancer is active.
- Blood tests are performed to check for general health and look for specific markers, such as elevated levels of lactate dehydrogenase (LDH), which can indicate a high tumor burden.
- A bone marrow aspiration and biopsy are performed to see if the cancer has reached the bone marrow.
- A lumbar puncture, or spinal tap, is done to collect cerebrospinal fluid and check for the presence of cancer cells.
Intensive Treatment Protocols
Due to the extremely rapid growth of Burkitt lymphoma, treatment must be initiated immediately after diagnosis. The standard of care involves short-term, high-intensity chemotherapy regimens that combine multiple drugs. Because chemotherapy drugs work best on cells that are dividing rapidly, they are particularly effective against this type of cancer.
Several established multi-agent chemotherapy protocols are used, with names like CODOX-M/IVAC and R-EPOCH. A common component in modern regimens is the addition of a monoclonal antibody called rituximab. Rituximab specifically targets a protein called CD20 on the surface of B-cells, including the cancerous ones, marking them for destruction by the immune system.
An important part of the treatment plan is central nervous system (CNS) prophylaxis. This involves administering chemotherapy directly into the cerebrospinal fluid, through a lumbar puncture or a device implanted under the scalp called an Ommaya reservoir. This preventative measure is taken to kill any cancer cells that may have spread to the brain and spinal cord, or to prevent them from getting there.
Prognosis and Life After Treatment
Despite its aggressive nature, Burkitt lymphoma has a high cure rate when treated with prompt and intensive therapy. The prognosis is particularly favorable for children, with long-term survival rates often reported to be between 80% and 90%. Cure rates for adults are also high, generally falling in the 70% to 80% range with modern treatment protocols.
Recovery from the intensive treatment can be a long process. The high doses of chemotherapy can cause significant short-term side effects and may lead to long-term health issues. Patients require careful monitoring during and after therapy to manage these effects.
Following the completion of treatment, long-term follow-up care is an important part of survivorship. These appointments involve regular check-ups and imaging scans to monitor for any signs of the cancer returning and to manage any late effects of treatment.