Burkitt Lymphoma and Burkitt’s-like Lymphoma: Differences

What Is Burkitt Lymphoma?

Burkitt lymphoma is a subtype of non-Hodgkin’s lymphoma involving B-cells. B-cells are immunological cells formed in the bone marrow and function against inflammation or infectious activity. It is a high-grade aggressive type of lymphoma that includes the excessive proliferation of B-cell lymphoma. Burkitt lymphoma was first described by a British surgeon Dr. Denis Burkitt in 1950 among children or young adults in Africa. It is mainly associated with children or young adults who may have developed malaria or the Epstein-Barr virus. The mechanism which is followed in the Burkitt lymphoma has an underlying disorder– malaria, which gradually weakens the immunity of the body and catches the Epstein Barr virus, which leads to the proliferation of B-cells into cancerous cells.

What Are the Types of Burkitt Lymphoma?

Burkitt lymphoma is a subtype of non-Hodgkin’s lymphoma. The World Health Organization (WHO) has classified Burkitt's Lymphoma into three types -

1. Endemic Type Burkitt Lymphoma - This type of lymphoma usually seen in African children affects their jaws and facial bones. It may affect extranodal organs like the gastrointestinal and kidneys and is also associated with the Epstein-Barr virus.

2. Sporadic Type Burkitt Lymphoma - Sporadic type of Burkitt lymphoma is observed worldwide, with 15 - 30 % of individuals associated with the Epstein-Barr virus.

3. Immunodeficiency Associated with Burkitt Lymphoma - Burkitt lymphoma is associated with disorders affecting the body's immune system especially related to HIV (Human Immunodeficiency Virus) or by the administration of immunosuppressive drugs.

What Are Burkitt-Like Lymphoma?

The difference between Burkitt and Burkitt-like lymphoma is not very evident. Burkitt-like lymphoma causes excessive proliferation of mature B-cells; it is a non-Hodgkin’s lymphoma. Burkitt-like lymphoma has similar clinical features and is associated with the disorder Burkitt lymphoma. Still, the cytoplasmic changes are seen as the size of the nucleus is larger than the Burkitt-like lymphoma.

What Is the Etiology of Burkitt Lymphoma and Burkitt-Like Lymphoma?

Burkitt lymphoma and Burkitt-like lymphomas can have various etiological factors -

1. Genetic Abnormalities -

• Genetic abnormalities cause Burkitt and Burkitt-like lymphoma because of the unusual rearrangement of the chromosome and molecules, leading to the translocation of genes and affecting the pathogenesis of lymphoma. The abnormalities are caused by the genes that cause MYC rearrangement.

2. Viruses -

• Epstein-Barr Virus (EBV) can cause high-grade Non-Hodgkin's lymphoma called — Burkitt's lymphoma, seen in all three subtypes of Burkitt's lymphoma.

• Human immunodeficiency virus (HIV) has late manifestations associated with non-Hodgkin's lymphoma.

• The Hepatitis C virus can affect the B-cells leading to the development of lymphoma.

• Helicobacter pylori are associated with gastric extranodal marginal zone B-cells lymphomas causing gastric mucosa-associated lymphoid tissue lymphoma (MALT).

4. Immunodeficiency -

• Immunodeficiency can be either congenital or acquired. In both cases, there is a predisposing factor for the Burkitt lymphoma subtype of Non-Hodgkin's lymphoma

• Examples of acquired immunodeficiency - HIV (Human immunodeficiency virus),

Iatrogenic immunosuppression.

What Are the Signs and Symptoms of Burkitt Lymphoma and Burkitt-Like Lymphoma?

The Burkitt lymphoma and Burkitt-like lymphoma are associated with malaria and the Epstein-Barr virus. They are classified into three types endemic, sporadic, and immunodeficient, which can also lead to other systemic or functional complications involving defects in jaws, bones, gastrointestinal tract, ovaries, breast, and kidneys or underlying causes like (HIV) Human Immunodeficiency Virus and administration of immunosuppressive drugs.

Following are the clinical features of Burkitt lymphoma and Burkitt-like lymphoma -

1. High-grade fever.

2. Loss of appetite.

3. Fatigue.

4. Night sweats.

5. Recurring Infections.

6. Headache.

7. Abdominal discomfort.

8. Joint pain.

9. Swollen Lymph nodes.

What Is the Difference Between Burkitt Lymphoma and Burkitt-Like Lymphoma?

The Burkitt lymphoma and Burkitt-like lymphoma are forms of non-Hodgkin’s lymphoma that involve aggressive proliferation of B-cells. Hematologists have observed that there are not very evident differences between Burkitt lymphoma and Burkitt-like lymphoma other than some morphological changes in the cytoplasm in the cells that have a larger nucleus in Burkitt lymphoma and a smaller nucleus in Burkitt-like lymphoma. Earlier Burkitt-like lymphoma was also classified as undifferentiated non-Burkitt lymphoma in modified Rappaport classification and small non-cleaved non-Burkitt lymphoma by National Science Institute. In 2001 World Health Organisation (WHO) introduced B-cell lymphoma with the aggressive proliferation of non-Hodgkin’s lymphoma in which the Burkitt lymphoma has two subtypes – Burkitt lymphoma with plasmacytoid differentiation and Burkitt-like lymphomas. Later it was cleared that Burkitt lymphoma and Burkitt-like lymphoma show different morphologies, genetic findings, and therapeutic implications.

What Are the Investigation for the Diagnosis of Burkitt Lymphoma and Burkitt-Like Lymphoma?

Burkitt lymphoma is an aggressive and high-grade lymphoma that causes the involvement of internal organs and spreads throughout the body. The lymphoma involves lymphadenopathy and extranodal organs.

Following are the investigations for the diagnosis of Burkitt lymphoma and Burkitt-like lymphoma.

1. Recording patient’s personal and family history.

2. Physical Examination of the patient involves inspections, palpitation of the enlarged lymph nodes, and auscultation.

3. Complete Blood Count - Complete blood count is a blood test that helps to determine the count of blood cells.

4. Bone Marrow Biopsy and bone marrow aspiration - A sample is collected from the bone marrow for examination.

5. X-rays Chest.

6. CT Scan (computerized tomography scan) - It is an imaging technique that helps to know the spread of lymphoma.

7. Lymph Nodes Biopsy - The enlarged lymph nodes are removed and collected for histopathological studies.

8. Renal Function Test - As kidneys also get involved in the Burkitt lymphoma, renal function tests are done to check the ability to function.

9. Liver Function Test.

10. Testing for HIV (human immunodeficiency virus)

11. Spinal fluid examination.

What Is the Management of Burkitt Lymphoma and Burkitt’s Lymphoma?

Burkitt Lymphoma and Burkitt-like lymphomas are aggressive and excessively proliferate, so early detection helps in a good prognosis of the disorder. However, non-Hodgkin’s lymphoma is a manageable type of lymphoma.

Treatment indicated for Burkitt and Burkitt-like lymphoma -

1. Chemotherapy is an ideal treatment advised for cancer and has a good prognosis.

2. Along with Chemotherapy, certain drugs are indicated for treating Burkitt's Lymphoma, like Rituximab, which attacks the cancer cells and stimulates the immune system.

3. Autologous stem cell transplantation.

4. Radiation Therapy.

5. Steroid Therapy.

6. Drugs involved in the treatment are - Cyclophosphamide, Cytarabine, Doxorubicin, Etoposide, Methotrexate, and Vincristine.

7. The supportive treatment.

Conclusion -

The Burkitt lymphoma and Burkitt-like lymphoma are subtypes of non-Hodgkin’s lymphomas that involve aggressive proliferation of B-cells with the association of malaria and Epstein-Barr virus. Burkitt lymphoma is a fatal disorder if it is left untreated; it mostly affects children and young adults. Early detection leads to a good prognosis with a 60 to 70 percent rate of survival.