Nguyen Jimmy T, Kini Ameet R, Li Yanxia, Sarvida Marie E, Manera Ricarchito
Departments of *Pediatrics †Pathology, Ronald McDonald Children's Hospital, Loyola University Chicago Health Sciences Division, Maywood, IL.
J Pediatr Hematol Oncol. 2014 Mar;36(2):e125-7. doi: 10.1097/MPH.0b013e318290b9e6.
Burkitt lymphoma (BL) is an aggressive, rapidly proliferating neoplasm of B-cell origin. A late recurrence should be investigated to differentiate whether it is a true relapse or a de novo lymphoma as this has therapeutic implications.
We report an HIV-negative white male individual, who at the age of 14 presented with recurrent BL in the abdomen occurring 6 years after successful treatment. Analysis of VDJ rearrangement showed marked dissimilarity in clonal peaks between the 2 tumors, suggesting that each tumor was associated with separate origins. The second tumor was treated as a de novo BL, and the patient remained in complete remission 2 years from recurrence without any evidence of the disease.
We present a case with 2 distinct BLs verified by the VDJ rearrangement analysis in a non-HIV infected individual. Our case supports the finding that a genetically discrete BL can be treated as if it were a new tumor, thereby reducing chemotherapeutic burden and treatment-related morbidity associated with the regimens for relapsed or refractory disease. Analysis of VDJ rearrangement seems to be a reliable assessment of tumor clonality.
