Burkitt Lymphoma - Signs, Symptoms, Diagnosis, and Treatment

Introduction:

The lymphatic system is one of the essential human body systems. This system has several lymph nodes at different body sites connected via tubes. These tubes, together with lymph nodes, are called the lymphatic system. The lymphatic system is responsible for the flow of a clear fluid called lymph. This clear liquid consists of two types of lymphatic cells: B lymphocytes and T lymphocytes. These are nothing but different white blood cells that fight infections if present. White blood cells are simply known to be the police force of the body. The function of B lymphocytes and T lymphocytes is to kill infectious organisms, but their role might vary slightly from each other.

What Is Burkitt Lymphoma?

Non-Hodgkin's lymphoma is a fast-growing type of cancer of the lymphatic system where abnormal amounts of incomplete WBC (white blood cells) are produced. When this happens, these WBCs are accumulated on different body parts. Burkitt lymphoma is a type of non-Hodgkin's lymphoma that affects the B lymphocytes. Hence, it is also called B-cell lymphoma. This cancer is a fast-growing type of cancer, and it has to be treated as soon as the diagnosis is made to prolong the lifespan of the individual.

What Are the Types of Burkitt Lymphoma?

The World Health Organization (WHO) classified Burkitt lymphoma into the following three types, namely -

1. Endemic Burkitt Lymphoma (African): This type of Burkitt lymphoma is prevalent among the children of Africa. The children affected are between the ages of four and seven. It affects boys more than girls.

2. Sporadic Burkitt Lymphoma (Non-African): This type of Burkitt lymphoma is a type that affects people globally other than in Africa. About one to two percent of lymphoma cases are Burkitt lymphoma worldwide. About 40 of pediatric lymphoma cases are Burkitt lymphoma in the US and Western Europe.

3. Immunodeficiency Associated Burkitt Lymphoma: This type of Burkitt lymphoma occurs in severely immunocompromised people. It is widespread in people with HIV (human immunodeficiency virus) infection as this virus makes the person immunocompromised. This type of Burkitt lymphoma can also be seen in people who have congenital immune disorders or if a person intakes immunosuppressant drugs for an extended period of time.

What Are the Signs and Symptoms Associated With Burkitt Lymphoma?

The symptoms of Burkitt lymphoma depend upon the type of Burkitt lymphoma. However, in cases of endemic Burkitt lymphoma, the following are the standard features seen:

• Endemic Burkitt lymphoma often starts as a tumor of the jaw or any of the facial bones.

• It can also involve the gastrointestinal tract, ovaries, and breasts.

• Cancer often invades the central nervous system (CNS), causing weakness, nerve problems, and paralysis.

• In cases of sporadic Burkitt lymphoma or immunodeficiency Burkitt lymphoma, the following are the common signs and symptoms:

• The tumor often starts as a huge mass in the abdomen and can spread to the liver, spleen, and bone marrow.

• This type of Burkitt lymphoma can also start from the ovaries or testes and spread to other vital structures like the brain and spinal fluid.

Some of the common symptoms of Burkitt lymphoma are discussed below -

• A rise in body temperature often occurs for no particular cause.

• Heavy sweating, especially at night.

• Heavy weight loss without any effort.

• Some might also experience itching throughout the body without any cause.

The doctors call the symptoms mentioned above B symptoms.

Burkitt lymphoma often starts or invades the abdomen and bowel, presenting with the following symptoms.

• Bowel blockage (obstruction) and bleeding caused due to it.

• Abdominal pain and discomfort.

• Abdominal swelling due to the accumulation of fluid in the abdomen.

• Back pain.

When Burkitt lymphoma affects the bone marrow, it can present with the following symptoms.

• Low blood cell counts.

• Tiredness.

• Shortness of breath.

How to Diagnose Burkitt Lymphoma?

Burkitt lymphoma is a fast-growing type of cancer, and therefore prompt diagnosis is crucial for successful management. Biopsy of the enlarged lymph nodes where a sample of the enlarged lymph node is seen under a microscope to confirm the diagnosis of Burkitt lymphoma.

• Computed tomography (CT) scan to check for the spread and extent of the tumor, and also aids in treatment of Burkitt lymphoma.

• Chest X-rays.

• Assessment of the spinal fluid.

• Bone marrow assessment.

• Blood tests.

What Is the Staging of Burkitt’s Lymphoma?

The St. Jude staging system is commonly used for pediatric patients, while the Ann Arbor system and Murphy staging system find widespread use in adults. The Ann Arbor system takes into account the patient’s symptoms, while the Murphy staging system is valued for its focus on extranodal disease and its ability to distinguish bone marrow involvement from central nervous system disease.

1. St. Jude/Murphy Staging System (Children)

• Stage I: Presence of a single tumor (extranodal) or a single anatomical area (nodal), excluding the mediastinum or abdomen. A tumor (extranodal) with regional node involvement on the same side of the diaphragm.

• Stage II: Presence of a single tumor (extranodal) with regional node involvement, lymph node involvement on the same side of the diaphragm (involving two or more nodal areas or two single extranodal tumors, with or without regional node involvement), or a primary gastrointestinal tract tumor (usually ileocecal) with or without associated mesenteric node involvement, grossly completely resected.

• Stage III: Presence of tumors on both sides of the diaphragm (involving two or more nodal areas or two single extranodal tumors), all primary intrathoracic tumors (e.g., mediastinal or pleural thymic), all extensive primary intra abdominal disease; unresectable abdominal disease, even if only in one area, or all primary paraspinal or epidural tumors, irrespective of other sites.

• Stage IV: In stage 4 Burkitt lymphoma,Any of the above stages with initial CNS or bone marrow involvement (only if less than 25 percent of the marrow is composed of Burkitt cells).

2. Murphy System (Adult)

• Stage I: A single nodal or extranodal site, excluding the mediastinum or abdomen.

• Stage II: Involvement of two or more nodal areas on one side of the diaphragm.

• Stage IIR: Abdominal disease that is completely resectable.

• Stage III: Presence of two or more nodal areas on opposite sides of the diaphragm, or a primary intrathoracic tumor, paraspinal or epidural tumors, extensive intra-abdominal disease.

  1. Stage IIIA: Abdominal disease that is completely non-resectable.

  2. Stage IIIB: Widespread multiorgan intra-abdominal disease.

• Stage IV: In stage 4 Burkitt lymphoma, involvement of the central nervous system or bone marrow.

Favorable stages include I or IIR.

3. Ann Arbor System (Adult)

• Stage I: Presence of a single nodal or extranodal site.

• Stage II: Involvement of two or more nodal areas on one side of the diaphragm, or localized presence in an extra-lymphatic site along with one or more sites on the same side of the diaphragm (IIE).

• Stage III: Presence of two or more nodal areas on opposite sides of the diaphragm, which may include or involve the spleen (IIIs), or localized presence in an extranodal site (IIIE).

• Stage IV: In stage 4 Burkitt lymphoma, diffuse or disseminated involvement of one or more extra-lymphatic sites, or the presence of two single extranodal tumors on opposite sides of the diaphragm.

Favorable stages include stages I, II, and III.

How to Treat Burkitt Lymphoma?

Burkitt lymphoma is a fast-growing type of non-Hodgkin's lymphoma and is fatal if left untreated. Therefore, the doctor starts the treatment as soon as the diagnosis is made to prolong the individual's lifespan.

• Intravenous chemotherapy, which often requires a hospital stay, is one of the standard treatment methods done for Burkitt lymphoma. In the treatment of Burkitt lymphoma, chemotherapy is the primary approach.

• Intrathecal chemotherapy is done where drugs are directly injected into the cerebrospinal fluid (CSF) when Burkitt lymphoma spreads to the brain and surrounding structures.

• Some drugs are used in various combinations to treat Burkitt lymphoma.

  1. Cytarabine.

  2. Cyclophosphamide.

  3. Etoposide.

  4. Methotrexate.

  5. Vincristine.

• Radiation therapy.

• Steroidal therapy.

In some cases as a part of treatment of Burkitt lymphoma, surgical removal of a part of the intestine might be done if obstruction or rupture occurs. Throughout the treatment of Burkitt lymphoma, close monitoring is essential to assess the patient's response to therapy and make any necessary adjustments.

Conclusion:

Burkitt lymphoma spreads fast to other vital organs of the body, which might be fatal. Among other stages, stage 4 Burkitt lymphoma represents an advanced and widespread form of the disease. Early diagnosis and appropriate treatment can increase survival rates in 60 % to 90 % of children. Treatment of Burkitt lymphoma may also include supportive therapies to manage side effects and complications associated with intensive chemotherapy. It can give a complete cure in children, whereas in adults the cure rate is variable. Recent advances have shown an increased life span.