Diagnosis and treatment of Burkitt lymphoma in adults: clinical practice guidelines from ERN-EuroBloodNet

Abstract

Burkitt lymphoma is a rare lymphoma entity that represents less than 5% of adult lymphomas. Although prognosis has improved with dose-dense therapy, Burkitt lymphoma remains an area of clinical and biological research with specificities due to the high incidence of CNS involvement and tumour lysis syndrome in patients with a high tumour burden. Few consensus recommendations are available concerning diagnosis, treatment, and prognostic factors in adult patients. In this Review, a European Reference Network on Rare Haematological Diseases (ERN-EuroBloodNet) expert panel has reviewed recent advances in the management of Burkitt lymphoma in the first-line setting to develop updated evidence-based and expert opinion-based recommendations on the management of this disease. The expert panel consisted of ten clinicians and pathologists involved in the clinical management of Burkitt lymphoma from eight EU member states. Additionally, two haematologists were included to support the systematic review process. A balanced representation was ensured between individuals affiliated and not affiliated with ERN-EuroBloodNet. Together with providing current indications on diagnosis and risk-adapted first-line therapy, the Review contains specific recommendations for the identification and management of important complications of Burkitt lymphoma such as tumour lysis syndrome and CNS-oriented therapy, and recommendations for prognostic assessment to guide treatment. Finally, unresolved questions for Burkitt lymphoma are highlighted, including questions around genetics, imaging, and second-line therapies, along with patient perspective.