Burkitt Lymphoma

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 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, particularly cytopenias, gastrointestinal effects, and tumor lysis and cytokine release syndromes. Patients may also have... 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... 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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