Answer: C. First line therapy for Burkitt lymphoma is hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone (R-hyper-CVAD).
Patients with the diagnosis of Burkitt lymphoma always warrant aggressive and immediate therapy with combination chemotherapy and aggressive intravenous hydration, urine alkalinization, and administration of allopurinol or rasburicase.
The combination chemotherapy consisting of rituximab plus hyperfractionated (rapidly cycled) cyclophosphamide, vincristine, doxorubicin, and dexamethasone (R-hyper-CVAD) is most appropriate for this patient with CD20-positive Burkitt lymphoma as seen in this patient. The most aggressive forms of large cell lymphoma include Burkitt lymphoma and lymphoblastic lymphoma. Onset of disease is acute, and patients usually present with life-threatening metabolic and structural abnormalities (this patient has early tumor lysis syndrome and impending airway obstruction). Treatment with R-hyper-CVAD, which is also used to treat acute lymphoblastic leukemia, is associated with high response rates (80%) and is curative in nearly 50% of patients with CD20-positive disease. The International Prognostic Index (IPI) score was developed to assist in determining prognosis before therapy. The IPI score is based on the patient's age, serum lactate dehydrogenase level, number of extranodal sites, disease stage, and performance status. Patients with Burkitt lymphoma have high IPI scores and always warrant aggressive and immediate therapy. Careful monitoring is required when treating patients with Burkitt lymphoma because rapid cell turnover and cell death are exacerbated by initiation of chemotherapy. Aggressive intravenous hydration, urine alkalinization, and administration of allopurinol or rasburicase are indicated in addition to chemotherapy.
Tracheostomy is not needed for this patient as he is not suffering from any form of airway obstruction. Therefore this is not appropriate.
Although Burkitt lymphoma can be localized in presentation, it is considered a generalized disease process and is treated with systemic agents. Therefore, neither surgery nor involved-field radiation therapy is indicated as primary treatment.
Because of their association with Helicobacter pylori infection, gastric mucosa-associated lymphoid tissue lymphomas can often be induced into complete and durable remission with the combination of antimicrobial agents and a proton pump inhibitor such as amoxicillin, clarithromycin, and omeprazole without the need for additional chemotherapy. This regimen is not effective for patients with aggressive large B-cell lymphoma, such as Burkitt lymphoma.
Reference
Molyneux EM, Rochford R, Griffin B, et al. Burkitt's lymphoma. Lancet. 2012 Mar 31;379(9822):1234-44. PMID: 22333947