Linfoma de Burkitt.

Resumo

Burkitt's lymphoma (BL) is a highly aggressive B-cell neoplasm characterized by the translocation and deregulation of the c-myc gene on chromosome 8. Three distinct clinical forms of BL are recognized: endemic, sporadic, and human immunodeficiency-associated. BL is a rapidly growing neoplasm requiring immediate diagnosis and treatment.We described and analyzed our experience with Burkitt's lymphoma (BL) diagnosis, treatment and outcome, during ten years.Retrospective study; clinical records of all children admitted with BL between 1st January 1998 and 31st December 2008 were analyzed. The following data were collected: age at admission, gender, clinical presentation, and time elapsed from initial complaints until diagnosis, disease localization, treatment and evolution.During the time period 21 children were admitted (19 boys), seven (33.3%) of which were diagnosed in 2008. The median age at diagnosis was seven years with a mean delay to diagnosis of 20,8 days (range 2-125 days). The most frequent site of primitive tumour was the abdomen (13), followed by tonsils (three), orbit (one), central nervous system CNS (two), tongue (one) and nasopharynx (one). The majority of patients in our study were presenting with a painfull abdominal mass. Diagnosis was established through tumour biopsy in 17 children, three by paracentesis or toracocentesis and one case was diagnosed only by genetic tests to the bone marrow. Genetic tests were positive in 11 patients. According to the Murphy classification, there were three stage II, 12 stage III and six stage IV tumours; 29% and 19% had bone marrow and central nervous system involvement, respectively. One child relapsed and was successfully treated with Rituximab® and autologous stem cell transplantation. The overall survival rate was 100%.