Treatment Approach
Forty-nine (89%) children underwent RIS. Forty-four (80%) received rituximab: 40 (91%) with concurrent chemotherapy and 8 (18%) with surgical resection. Eleven (20%) children, 6 (55%) with non-B cell disease, did not receive rituximab: 5 received chemotherapy only, 1 received chemotherapy and radiation, 1 underwent RIS only, 2 were managed with surgical resection only, and 2 received no treatment due to instability. No patient received EBV-CTLs as treatment for first presentation of monomorphic PTLD.
Forty-six (84%) children had a chemotherapy component to their treatment plan. Twenty-two (48%) were treated according to the LMB-96 protocol and 14 (30%) received low-dose cyclophosphamide with prednisone, all low-dose cyclophosphamide with prednisone patients also received rituximab (CPR).18,19,31 Of the 22 children receiving LMB96, 17 had Burkitt lymphoma and 5 DLBCL. Of the 14 receiving CPR, 6 had Burkitt lymphoma and 8 DLBCL. Chemotherapy received by the other 10 (22%) children included: T-cell ALL therapy n=4, cyclophosphamide/vincristine n=2, methotrexate/cytarabine n=2, cyclophosphamide/ vincristine/methotrexate n=1, and Hodgkin lymphoma therapy n=1.
Fifteen children (11 with Burkitt/DLBCL and 4 with NK/T cell lymphoma) required further lines of treatment, 11 (73%) for relapse and 4 (27%) for refractory disease. Their initial treatment was not uniformly conservative: 1 received single-agent rituximab, 3 CPR, 5 LMB96 chemotherapy, and 6 received other multiagent chemotherapy.