(See also Overview of Lymphoma Overview of Lymphoma Lymphomas are a heterogeneous group of tumors arising in the reticuloendothelial and lymphatic systems. The major types are Hodgkin lymphoma Non-Hodgkin lymphoma See table Comparison of Hodgkin... read more and Non-Hodgkin Lymphomas Non-Hodgkin Lymphomas Non-Hodgkin lymphomas are a heterogeneous group of disorders involving malignant monoclonal proliferation of lymphoid cells in lymphoreticular sites, including lymph nodes, bone marrow, the... read more .)
Classic Burkitt lymphoma is endemic in central Africa and constitutes 30% of childhood lymphomas in the US. The form endemic to Africa often manifests as enlargement of the jaw or facial bones.
In sporadic (non-African) Burkitt lymphoma, abdominal disease predominates, often arising in the region of the ileocecal valve or the mesentery. Tumor may cause bowel obstruction. Extranodal sites such as the brain or other solid organs may also be involved. In adults, disease may be bulky and generalized, often with massive involvement of liver, spleen, and bone marrow. Central nervous system (CNS) involvement is often present at diagnosis or with relapsing lymphoma.
Burkitt lymphoma is the most rapidly growing human tumor, and pathology reveals a high mitotic rate, a monoclonal proliferation of B cells, and a “starry-sky” pattern of benign macrophages that have engulfed apoptotic malignant lymphocytes. On FDG-PET (fluorodeoxyglucose-positron emission tomography) scans, the tumors are highly metabolic. There is a distinctive genetic translocation involving the C-myc gene on chromosome 8 and the immunoglobulin heavy chain of chromosome 14. The disease is closely associated with Epstein-Barr virus infection Infectious Mononucleosis Infectious mononucleosis is caused by Epstein-Barr virus (EBV, human herpesvirus type 4) and is characterized by fatigue, fever, pharyngitis, and lymphadenopathy. Fatigue may persist weeks or... read more in endemic lymphoma; however, it is uncertain whether Epstein-Barr virus plays an etiologic role. Burkitt lymphoma occurs frequently in patients with HIV/AIDS and is an AIDS-defining disease .
Diagnosis of Burkitt Lymphoma
Lymph node or bone marrow biopsy
Histopathologic diagnosis is based on biopsy of lymph node or tissue from another suspected disease site, such as the bone marrow. Rarely, laparoscopy may be used for both diagnosis and treatment.
Staging studies must be expedited because the tumor grows rapidly. Staging includes fluorodeoxyglucose (FDG)-positron emission tomography (PET)/CT tumor imaging; if unavailable, CT of the chest, abdomen, and pelvis may be done instead. Patients should also have bone marrow biopsy, cerebrospinal fluid cytology, and laboratory studies to include lactate dehydrogenase (LDH).
Treatment of Burkitt Lymphoma
Treatment must be initiated rapidly because these tumors grow rapidly. An intensive alternating regimen of cyclophosphamide, vincristine, doxorubicin, methotrexate, ifosfamide, etoposide, and cytarabine (CODOX-M/IVAC) plus rituximab results in a cure rate of > 80% for children and adults < 60 years. For patients > 60 years, regimens such as rituximab plus etoposide, prednisone, vincristine (Oncovin), and doxorubicin (dose-adjusted R-EPOCH) are also commonly used with success. For patients without CNS metastases, CNS prophylaxis (eg, with systemic and/or intrathecal methotrexate and/or cytarabine) is essential.
With treatment, tumor lysis syndrome Tumor Lysis and Cytokine Release Syndromes Adverse effects are common in patients receiving any cancer therapy. Patients may also have adverse effects resulting from their cancer. Successfully managing these adverse effects is important... read more is common, and patients must receive IV hydration, allopurinol often with rasburicase, alkalinization of the urine (in the absence of hyperphosphatemia), and close attention to electrolytes (particularly potassium, phosphorus, and calcium). Rasburicase is contraindicated in patients with G6PD deficiency Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an X-linked enzymatic defect common in people with African ancestry that can result in hemolysis after acute illnesses or intake of oxidant... read more because it may cause hemolytic anemia in these patients. Some patients may require dialysis Overview of Renal Replacement Therapy Renal replacement therapy (RRT) replaces nonendocrine kidney function in patients with renal failure and is occasionally used for some forms of poisoning. Techniques include continuous hemofiltration... read more for hyperkalemia Hyperkalemia Hyperkalemia is a serum potassium concentration > 5.5 mEq/L (> 5.5 mmol/L), usually resulting from decreased renal potassium excretion or abnormal movement of potassium out of cells. There are... read more .
If the patient presents with bowel obstruction secondary to tumor but the tumor is completely resected at initial diagnostic-therapeutic laparotomy, then aggressive therapy is still indicated, but fewer cycles may be needed. Patients at the end of treatment should have a complete metabolic response documented by PET or a complete response documented by CT scan and bone marrow biopsy. Outcome is poor in the 20% of patients in whom induction fails or relapse (typically in the first 12 months) occurs. Salvage therapy or clinical trials should be considered.
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