Linfoma di Burkitt in età pediatrica

Abstract

Sporadic Burkitt lymphoma (BL) accounts for about 80% of non-Hodgkin lymphomas in childhood and adolescence, with an incidence of 3-5 cases per million in developed countries. The hallmark of LB is the deregulation of MYC oncogene, usually caused by the [t(8;14)(q24;q32)] translocation, involving the gene of immunoglobulin heavy chain. BL is a very aggressive neoplasm, with a doubling time of 12-24 hours and its diagnosis and therapy are a true haematologic emergency. Abdomen, head and neck are the most frequent sites of presentation and the involvement of ileo-caecal area can cause an intussusception as first manifestation of the disease. <br> BL and mature B-cell acute leukaemia (the leukaemic manifestation of BL) share the same therapeutic approach, intensive short courses with multiple alternating chemotherapy agents, the use of high dose-methotrexate and intrathecal CNS prophylaxis. The outcomes are satisfactory: in Italy during the period 1997-2014 the children with BL were treated according to the AIEOP LNH-97 protocol, achieving more than 90% of 5 year-survival. <br> In the last decades, the prognosis for children and adolescents with cancer has dramatically improved and the increasing number of long-term survivors has focused the attention of clinicians on the late effects of cancer and its therapy. A multidisciplinary follow up and a correct transition from the paediatrician to the primary care doctor plays a central role not only in the early detection and therapy of late effects of cancer but also in supporting the patient to realize their physical, mental and social well-being as much as possible.