Characteristics
Burkitt lymphoma (BL) is a rare, aggressive form of non-Hodgkin lymphoma (NHL) that arises from B-lymphocytes, a type of white blood cells that produce antibodies. BL is the most common form of NHL in children, but it can also develop in adults. BL is more common in males than in females. BL follows an aggressive disease course and can be fatal. However, when treated timely, most patients can obtain a remission.
There are three different types of BL. The most common form on a global level consists of endemic BL, which typically affects children living in central Africa. The development of endemic BL is linked to the Epstein-Barr virus and children with this disease often develop tumors in their jaws or facial bones. The most common BL subtype in the Western world is referred to as sporadic BL. This type of BL usually affects children between the ages of 3 and 12. Finally, immunodeficiency-related BL is mainly found in patients with HIV, and (to a lesser extent) in patients with a compromised immune system due to other causes.
In most patients with BL, the cause of the disease is unknown. As indicated before, there is a relationship between infections with the Epstein-Barr virus and the development of BL, but this does not mean that everyone who has this virus will develop BL. In addition to this, researchers have established that many BL patients harbor a mutation in the gene that produces the MYC protein, which has an important role in the control of the cell growth.
Symptoms
Symptoms of BL usually develop very quickly, over just a few days or weeks. The most common symptom consists of the development of lumps (swollen lymph nodes) in different parts of the body. These lumps can double in size within hours.
Sporadic BL often grows in the abdomen and bowel, leading to abdominal pain, nausea, and a swelling of the belly. Furthermore, BL can cause a bowel blockage (obstruction) or cause bleeding in the bowel.
Other common symptoms of BL include heavy sweating (especially at night), the development of fever without an obvious cause and unexplained weight loss over a short period of time. These three symptoms often occur together and are known as ‘B-symptoms’. In addition to this, patients with BL may develop a (severe), generalized itch.
In BL patients with bone marrow involvement (about a third of patients), a disturbed blood cell production may lead to shortness of breath and fatigue, an increased risk of infections and a tendency to bleed or bruise easily.
Finally, BL can also develop in the head and neck area, or in other parts of the body, such as the chest, breasts, spleen, or central nervous system (brain and spinal cord). The development of bulky lymphoma masses in these body parts can lead to a wide range of symptoms.
Diagnostic tests
The diagnosis of BL starts with a physical examination, during which the doctor palpates lymph nodes and other areas of the body to check for swelling. The doctor will also assess the medical history of the patient to better understand the general health status and any potential risk factors.
If a BL is suspected, a lymph node biopsy is performed to make a final diagnosis. This is a simple procedure during which all, or part of a suspicious lymph node is removed from the body and sent to the laboratory for a microscopic evaluation. If this analysis is indicative for BL, additional tests can be performed to assess how far the cancer has spread. This includes blood tests, a bone marrow biopsy and imaging tests to look for signs of lymphoma in other areas of the body (X-ray, CT, PET, MRI, ultrasound). When there is a suspicion for central nervous system involvement, patients may also undergo a lumbar puncture (a procedure to take a sample of fluid from your spine) and an MRI scan.
Treatments
Given the aggressive disease course of BL, treatment is usually initiated immediately after the diagnosis. Most treatments for BL are very intensive and can last several months. BL is usually treated with a number of different chemotherapy drugs in combination with a monoclonal antibody called rituximab (chemoimmunotherapy).
Given the tendency of BL to spread to the central nervous system, patients may also receive additional treatment to prevent this. This treatment consists of chemotherapy injected directly into the fluid that covers the spinal cord.
When lymphoma masses press on certain organs, or cause obstructions, patients may also receive radiotherapy or undergo surgery to remove the mass in question.
While BL usually stays in remission after this intensive first line therapy, some patients may suffer a relapse, or may have a suboptimal treatment response (refractory disease). Unfortunately, relapsed, or refractory BL can be difficult to treat. In most cases, a new course of chemoimmunotherapy (with different chemotherapy agents) is used in these patients. If patients respond well to this new course of chemotherapy and are fit enough, doctors may opt for a stem cell transplantation.
Finally, by participating in a clinical trial, patients may have the option to be treated with new treatment modalities.
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