Burkitt Lymphoma: Types, Signs, Symptoms, Stages, Treatment, Survival Rate, Prognosis

The lymphatic system is a part of the immune system which filters and diminishes the viruses, bacteria and other infective cells from the body. It consists of various organs like lymph glands, lymph nodes, bone marrow, spleen and thymus.

The lymphocytes in the lymphatic system play a vital role in fighting against infection. These are divided into two types of cells-

• T-cells-The cells that grow in the thymus gland.
• B-cells-The cells that grow in the bone marrow.

When these cells grow in an uninhibited way they result into a disorder called Lymphoma. Non-Hodgkin lymphoma is a cancer in the lymphatic system. The type of non-Hodgkin lymphoma is classified on the cell type and their uninhibited growth.

Burkitt Lymphoma, introduced by Denis Parsons Burkitt, a surgeon in 1958, is a type of non-Hodgkin lymphoma.

The rare type of non-Hodgkin lymphoma in which the growth of B-cell lymphocytes exceeds is called Burkitt lymphoma. Burkitt lymphoma is a rapid growing disorder commonly found in men.

Burkitt lymphoma can be classified into three types. The classification is based on the variants. However, morphologically it is difficult to identify each of them, due to its similarity. The types are:

• Endemic Burkitt Lymphoma- This type of Burkitt lymphoma affects children who are suffering from malaria, especially in endemic regions like Africa. Endemic Burkitt Lymphoma is associated to minimal resistance to a virus called Epstein-Barr virus (EBV). Glandular Fever is caused by the EBV virus. The endemic Burkitt lymphoma is also known as African lymphoma. It affects distal ileum, jaw bone, breasts, ovaries, cecum or kidneys.
• Sporadic Burkitt Lymphoma- This type of Burkitt lymphoma is closely associated to the cancer cells of endemic Burkitt lymphoma. In rare instances, the sporadic Burkitt lymphoma is associated to the EBV virus. The ileocecal region is associated with this type of Burkitt lymphoma. It is commonly found in UK and is also known as non-African type.
• Immunodeficiency Burkitt Lymphoma- This type of Burkitt lymphoma is commonly found in patients whose immune system is suppressing due to certain type of drugs or have undergone an organ transplant surgery. The initial stages of HIV or AIDS may be linked to Immunodeficiency Burkitt lymphoma.

• DLBCL- Diffuse large B cell lymphoma
• B-cell lymphoma- which looks like Burkitt lymphoma but has no significant effect on MYC gene
• Double-hit lymphoma- it aggressively alters the arrangements of genes that are responsible for cell growth

These lymphomas are rare and are often treated like a typical Burkitt lymphoma.

Burkitt lymphoma is commonly associated to HIV infection. 1 in 6 patients of lymphoma have HIV infection. This is seen prior to immunodeficiency in the body.

The HIV-Linked type is difficult to treat due to higher risk of infections.

30-50% of children have a higher chance of getting non-Hodgkin lymphoma including Burkitt lymphoma. The children of age between 5-10 years have a higher risk of Burkitt lymphoma. In the endemic Burkitt lymphoma, the jaw bone is affected. Moreover, the permanent teeth formation may get affected in children.

The signs and symptoms of Burkitt Lymphoma may develop quickly as the lymphoma cells grow significantly. This may result into lymphoma at many places.

The common signs and symptoms of Burkitt Lymphoma include:

• Swelling in various parts of the body- as the lymph nodes grow.
• Critical Bowel problems- bleeding from bowel or bowel obstruction. Burkitt lymphoma has a significant impact on bowel.
• Abdominal pain and abdominal swelling is also a sign or symptom of Burkitt Lymphoma- usually due to collection of ascites and diarrhea.
• Extranodal lymphomas-these includes sites in other parts of the body like kidneys, liver, breasts and ovaries apart from the lymphatic system and the lymph nodes.

The other symptoms of Burkitt Lymphoma include:

• Fatigue
• Night sweat
• Loss of appetite
• Weight loss
• Fever
• Anemia
• Shortness of Breath
• Low blood counts in bone marrow
• Headaches
• Lack of concentration
• Siezures

The stages of Burkitt lymphoma are identified by the doctors during diagnosis through staging. The staging system helps in recognizing the affected group of lymph nodes and the other organs in the body. The lymph nodes below or above the diaphragm are assessed. There are two types of staging systems which are separately used for adults and children. They are:

• St Jude/Murphy Staging System: The type of staging system used for staging lymphomas in teenagers and children.
• Ann Arbor Staging System: The commonly used staging system for most type of lymphomas, especially in adults.

The stages of Burkitt lymphoma are classified into four stages and are marked in roman numbers. They are:

• Stage I of Burkitt Lymphoma- This is the primary stage of BL, where one lymph node group or an extranodal site is affected.
• Stage II of Burkitt Lymphoma – In this stage, BL has affected two or more lymph nodes groups on the same side of diaphragm.
• Stage III of Burkitt Lymphoma – The stage which has affected both the sides of diaphragm i.e. below and above including multiple sites like extranodal site and group of lymph nodes.
• Stage IV of Burkitt Lymphoma – The critical stage of BL, which spreads outside the lymphatic system i.e. bone marrow, lungs, liver and the central nervous system.

The staging system helps in understanding the severity of Burkitt Lymphoma and helps the doctor in deciding its treatment.

The children are at higher risk than adults to get Burkitt lymphoma. Moreover, it is commonly found in males than in females. The regions where there is a higher occurrence of Burkitt lymphoma is Africa, Papua New Guinea, South America and the Middle East.

EBV virus may contribute to Burkitt lymphoma. Moreover, tumor growth due to herbal extracts and various viral-infections may cause Burkitt lymphoma.

The survival rate of Burkitt lymphoma can be up to 71 percent under intensive care and therapy. The chemotherapy has a significant impact on the patients of Burkitt lymphoma. However, the survival rate would be low if the cancer would infect bone marrow or cerebrospinal fluid.

The pathophysiology of Burkitt lymphoma (BL) is not well defined. However, some of the causes of Burkitt lymphoma include:

• Malaria infection
• Epstein-Barr virus (EBV)
• Activation of C-my gene.

The diagnosis of Burkitt lymphoma can be done with the help of following tests:

• Computed Tomography (CT) scan- It is a vital imaging technique to measure the Burkitt lymphoma in various areas of chest, pelvis and abdomen.
• Complete blood count (CBC)- To measure the infection of Burkitt lymphoma in the blood.
• Chest X-ray- To see the infection or the growth of tumor of Burkitt lymphoma in the chest.
• Bone-marrow Biopsy- To test the growth of lymph nodes in bone marrow.
• PET scan- It is an imaging technique to see the Burkitt lymphoma infection in the body.
• Lymph node biopsy- To test the growth and type of lymph nodes.
• Examination of Cerebrospinal fluid (Lumbar Puncture)- To check the infection of Burkitt lymphoma in the central nervous system (CNS).

The primary treatment of Burkitt lymphoma can be done with the help of chemotherapy. Intrathecal chemotherapy can be given to minimize the infection in cerebrospinal fluid in the CNS.

The other treatments that can be given to treat Burkitt lymphoma are:

• Immunotherapy- It is a therapeutic intervention that fights the Burkitt lymphoma with the help of the immune system. It helps in destructing the exceeding B-cells from the body.
• Stem Cell Transplant- It is used to transplant bone marrow, which is highly affected by the lymphatic cells. It is also known as bone marrow transplant.
• Radiotherapy- It is a significant tool to destroy the cancerous B-cells from the body using high energy radiation.
• Steroid Therapy- It helps in killing the cells and is usually administered along with the chemotherapy.

Surgery for Burkitt Lymphoma- It is done to remove the tumor or affected tissue.

The complications of Burkitt lymphoma include:

• Tumor lysis Syndrome- A syndrome occurring at the initial stage of chemotherapy destroying the tumor cells. This happens due to an accelerating growth of B-cells.
• Rapid tumor growth in abdomen leads to its obstruction.
• Cardiomyopathy, disease of the heart muscle which is related to anthracycline, the drug used for cancer treatment.
• Bowel infection.
• Infections during neutropenia.
• Obstruction in uropathy.
• Secondary leukemias.
• Renal failure due to improper filtration in the kidneys.

Burkitt Lymphoma, though prevalent in specific areas of the world should be treated immediately and extensively once diagnosed as it could spread to the entire body very fast through the lymph node and lymphatic system. Since even malaria infection could cause it, any symptoms of Burkitt lymphoma post malaria should be taken seriously and medical advice must be taken.