What is Burkitt's Lymphoma?
Burkitt's Lymphoma is a highly aggressive B-cell non-Hodgkin lymphoma. It is characterized by the rapid growth of tumors in the lymphoid tissue, particularly affecting children and young adults. This malignancy is named after Denis Burkitt, who first described the disease in African children in the 1950s.
Histological Features
Histologically, Burkitt's Lymphoma is identified by its "starry sky" appearance. This pattern is due to the presence of numerous apoptotic bodies, which are engulfed by benign macrophages, creating the "stars" against a background of densely packed malignant lymphocytes, the "sky". The malignant cells are medium-sized with round nuclei, multiple nucleoli, and a high mitotic index.Cell Origin and Genetic Abnormalities
Burkitt's Lymphoma originates from
germinal center B-cells. One of the hallmark genetic features is the translocation involving the
MYC gene, commonly t(8;14)(q24;q32), which places the MYC gene under the control of the immunoglobulin heavy chain enhancer, leading to uncontrolled cell proliferation.
Role of Epstein-Barr Virus (EBV)
The endemic variant of Burkitt's Lymphoma, commonly found in Africa, is almost always associated with
Epstein-Barr Virus (EBV) infection. EBV plays a role in the pathogenesis by promoting B-cell proliferation and survival, which contributes to the oncogenic process.
Clinical Presentation
Patients with Burkitt's Lymphoma often present with rapidly growing masses. In the endemic form, jaw or facial bone involvement is common, whereas the sporadic form often presents with abdominal tumors. Other symptoms may include fever, night sweats, and weight loss.Diagnostic Techniques
The diagnosis of Burkitt's Lymphoma relies on a combination of histological examination, immunophenotyping, and genetic studies. Immunohistochemistry typically shows positivity for
CD20,
CD10, and
BCL6, and negativity for
BCL2. Fluorescence in situ hybridization (FISH) or polymerase chain reaction (PCR) can be used to detect MYC translocations.
Treatment and Prognosis
Burkitt's Lymphoma is highly aggressive but also highly responsive to intensive chemotherapy regimens. The prognosis is generally good with appropriate treatment, especially in pediatric cases. However, the prognosis can be poorer in adults and those with advanced disease at diagnosis.Conclusion
Burkitt's Lymphoma is a unique and highly aggressive form of lymphoma with distinct histological features and genetic abnormalities. Understanding its histological and molecular characteristics is crucial for accurate diagnosis and effective treatment.
