Burkitt's Lymphoma: A Malignant Disease of B-cell Origin
Introduction
Burkitt's lymphoma (BL) is an aggressive type of non-Hodgkin lymphoma (NHL) characterized by rapidly proliferating B-cells. It was first described by the Irish surgeon Denis Burkitt in 1958 and is commonly found in equatorial Africa, where it is the most common childhood malignancy.
Etiology and Pathogenesis
The exact cause of BL is unknown, but it is believed to be associated with the Epstein-Barr virus (EBV). EBV is a ubiquitous herpesvirus that infects most humans worldwide, but only a small proportion develop BL. It is hypothesized that in individuals with certain genetic predispositions, such as mutations in the c-myc proto-oncogene, EBV infection can trigger the development of malignant B-cells.
Clinical Features
BL presents with various clinical manifestations depending on the location and extent of the disease.
- Jaw involvement: Bilateral facial swelling with bony destruction is a common presenting symptom.
- Abdominal involvement: Abdominal pain, distension, and ascites can occur due to infiltration of the gastrointestinal tract and mesentery.
- Central nervous system (CNS) involvement: CNS disease is found in approximately 30% of patients, presenting with symptoms such as seizures, cranial nerve palsies, or altered mental status.
- Other sites: BL can also involve lymph nodes, bone marrow, skin, and other organs.
Diagnosis
Diagnosis of BL requires a combination of clinical examination, imaging studies, and pathological confirmation.
- Clinical examination: Physical examination reveals enlarged lymph nodes, facial swelling, or other manifestations of the disease.
- Imaging studies: Computed tomography (CT) and magnetic resonance imaging (MRI) scans can identify the extent and location of the lymphoma.
- Pathological confirmation: Biopsy of the affected tissue is essential for confirming the diagnosis. Histopathology typically shows a diffuse proliferation of medium-sized B-cells with a starry-sky appearance due to interspersed macrophages.
Treatment
BL is highly curable with early diagnosis and aggressive treatment. The standard treatment regimen involves a combination of chemotherapy, targeted therapy, and supportive care.
- Chemotherapy: CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) is the most commonly used chemotherapy regimen for BL.
- Targeted therapy: Rituximab, a monoclonal antibody that targets the CD20 antigen on B-cells, is often added to chemotherapy for improved outcomes.
- Supportive care: Blood transfusions, antibiotics, and pain management are essential aspects of supportive care.
Prognosis
The prognosis of BL depends on various factors, including the stage of the disease at diagnosis, patient age, and response to treatment. With early diagnosis and appropriate treatment, the overall survival rate for BL can approach 80%. However, relapsed or refractory disease is associated with a worse prognosis.
Conclusion
Burkitt's lymphoma is an aggressive B-cell lymphoma with a unique association with EBV. Despite its aggressive nature, it is highly curable with early diagnosis and prompt initiation of appropriate treatment. Advances in chemotherapy and targeted therapies have significantly improved the outcomes for patients with BL. Ongoing research continues to explore novel approaches to improve the diagnosis, treatment, and prognosis of this disease.