High Grade B-Cell Lymphoma Burkitt-like Lymphoma

High grade B-cell lymphoma Burkitt-like lymphoma is a rare and extremely fast-growing type of cancer that affects the body’s infection-fighting system, sharing features with both Burkitt lymphoma and other aggressive B-cell cancers while lacking specific genetic markers that define these diseases.

Table of contents

• Understanding High Grade B-Cell Lymphoma
• Relationship to Burkitt Lymphoma
• Symptoms and Warning Signs
• Diagnosis and Testing
• Treatment Approaches

Understanding High Grade B-Cell Lymphoma

High grade B-cell lymphoma (HGBCL) is a type of aggressive non-Hodgkin lymphoma, which means it is a cancer that affects the lymphatic system—the network of organs, vessels, and tissues that help your body fight infections^[8]. The term “high grade” indicates that this cancer grows very quickly and needs immediate treatment.

HGBCL develops from abnormal B lymphocytes (also called B cells), which are white blood cells that normally help protect your body from disease^[8]. When these cells become cancerous, they multiply rapidly and can spread throughout the body. This type of lymphoma shares characteristics with other aggressive B-cell cancers, particularly Burkitt lymphoma and diffuse large B-cell lymphoma^[8].

The World Health Organization recognized HGBCL as its own distinct category in 2016 because research showed it differs in important ways from other types of B-cell lymphomas^[8]. About 5 percent of cases that look like diffuse large B-cell lymphoma and between 32 to 78 percent of cases that appear to be Burkitt lymphoma actually have the genetic features of HGBCL^[8].

Relationship to Burkitt Lymphoma

Burkitt-like lymphoma is closely related to Burkitt lymphoma but has some key differences. Burkitt lymphoma is one of the fastest growing cancers in humans, with cancer cells doubling approximately every 25 hours^[6]. It is characterized by a specific genetic change called a translocation (rearrangement) involving the MYC gene^[2].

HGBCL is characterized by rearrangements in two particular genes: one involves the MYC gene, and the other involves either the BCL2 gene or, less commonly, the BCL6 gene^[8]. This combination of genetic changes makes HGBCL distinct from typical Burkitt lymphoma, even though the diseases can look very similar under the microscope.

In adults, Burkitt lymphoma is often hard to tell apart from diffuse large B-cell lymphoma, making accurate diagnosis especially important because these conditions are treated differently^[2]. For this reason, doctors strongly recommend that patients get an opinion from a lymphoma expert^[2].

Burkitt lymphoma itself comes in three main forms: endemic (most common in Africa and linked to malaria), sporadic (found worldwide), and immunodeficiency-related (affecting people with weakened immune systems)^[3]. All three forms are equally aggressive despite their different causes and locations in the body.

Symptoms and Warning Signs

Because HGBCL and Burkitt-like lymphoma grow extremely rapidly, symptoms often appear suddenly and get worse very quickly—sometimes within just a few days^[2]. The specific symptoms depend on where in the body the lymphoma develops.

Common symptoms include a rapidly growing tumor or mass, often in the abdomen, and swollen lymph nodes in the neck, armpit, or groin^[2]. Many people experience abdominal pain or swelling, nausea, vomiting, or changes in bowel habits^[2]. The disease may affect the jaw, central nervous system (brain and spinal cord), bowel, kidneys, ovaries, or other organs^[2].

General symptoms that doctors call “B symptoms” include unexplained weight loss (more than one-tenth of your total body weight), fever and night sweats, fatigue or weakness, and decreased appetite^[2]. Some people also experience loss of appetite and extreme tiredness^[4].

If the lymphoma grows in the bone marrow (the soft tissue inside bones where blood cells are made), it can crowd out normal blood cells, leading to tiredness, shortness of breath, bleeding, and easy bruising^[4]. The disease can also spread to the central nervous system^[2].

Because these lymphomas advance so rapidly, early detection and prompt medical evaluation are critical^[2]. Anyone experiencing these symptoms should speak with a healthcare provider immediately.

Diagnosis and Testing

Diagnosing HGBCL and Burkitt-like lymphoma requires several tests and procedures. The process often begins with a physical exam to check for swollen lymph nodes and a neurological exam to test how well the brain and nerves are working^[12].

Blood tests can sometimes show whether lymphoma cells are present and measure levels of lactate dehydrogenase (LDH), an enzyme that is often higher in people with lymphoma^[12]. Blood tests can also check for infections that may increase the risk, such as Epstein-Barr virus and HIV^[12].

Imaging tests create pictures of the body to show the location and extent of the lymphoma. These might include CT scans (computed tomography) and PET scans (positron emission tomography)^[12]. Doctors may also perform an MRI (magnetic resonance imaging) if they think the cancer is affecting the central nervous system^[12].

A lymph node biopsy is often necessary to look for cancer cells. This procedure removes all or part of a lymph node for testing in a laboratory^[12]. Bone marrow aspiration and biopsy collect cells from the bone marrow, usually from the hip bone, to check whether the lymphoma has spread there^[12].

Special molecular tests that check for gene rearrangements in chromosomes under a microscope are used to confirm a diagnosis of HGBCL and distinguish it from other types of lymphoma^[8]. These tests are essential because HGBCL, standard Burkitt lymphoma, and diffuse large B-cell lymphoma can look very similar but require different treatment approaches^[11].

Treatment Approaches

Treatment for HGBCL and Burkitt-like lymphoma must begin quickly because these cancers grow so rapidly. Patients should be admitted to the hospital for fast diagnosis and immediate treatment^[10].

Chemotherapy (medicines that kill cancer cells) is the main treatment^[10]. Treatment usually includes intensive combination chemotherapy together with a targeted drug called rituximab, which specifically attacks B cells. Doctors call this combination chemoimmunotherapy^[4].

One major concern during treatment is tumor lysis syndrome, a dangerous condition that can occur when cancer cells die very quickly and release their contents into the bloodstream. This is especially likely with fast-growing tumors^[10]. Patients receive preventive measures including medicines to protect the kidneys, aggressive hydration with intravenous fluids, and close monitoring of blood chemistry^[10].

Surgery and radiation therapy generally have no role in treating these lymphomas^[10]. However, doctors may use other supportive treatments as needed, such as antibiotics for infections, growth factors to help the body produce blood cells more quickly, and blood transfusions for anemia or bleeding problems^[10].

Clinical trials—research studies testing new treatments—are strongly encouraged because doctors are still working to establish the best treatment approach for these rare cancers^[10]. Recent research using a regimen called dose-adjusted EPOCH-R has shown encouraging results in treating HGBCL^[11].

Treatment for these aggressive lymphomas can be very intensive and may cause significant side effects. However, when diagnosed and treated promptly, many patients can achieve complete remission (no signs of cancer)^[1]. The prognosis is generally better in children than in adults^[3]. Despite the aggressive nature of these diseases, significant numbers of patients can be cured with intensive treatment^[14].