Post-transplant lymphoproliferative disorders in children: The role of chemotherapy in the era of rituximab

Abstract

PTLD are the most frequent neoplasms in children postorgan transplantation. We describe our experience in the treatment of 14 children (three with early and 11 with late-onset disease) treated with a step-wise protocol developed at our institution. Treatment consisted of reducing immunosuppressants, followed by rituximab and chemotherapy if required. Rituximab, incorporated into the protocol in 2001, has been determinant for the total chemotherapy burden patients need to achieve remission. In seven patients who did not receive rituximab, anthracycline total dose ranged from 160 to 240 mg/m(2), while only one of the patients receiving rituximab required DOXO (range: 0-120 mg/m(2)) (p = 0.003). The use of alkylating agents was also notably lower in patients receiving rituximab (median dose = 1200 mg/m(2)) compared with those who did not receive rituximab (median dose = 5800 mg/m(2)) (p = 0.006). Twelve patients are in remission and two died, one from refractory disease and the other from septic shock. Two-year OS and EFS were 85.7% and 57%, respectively. In conclusion, our experience with the use of rituximab in children with PTLD after solid organ transplantation appeared to be associated with a lesser requirement for alkylating agents and anthracyclines compared with historical subjects, suggesting a reduction in the side effects of these agents.

18F-FDG PET/CT in the management of patients with post-transplant lymphoproliferative disorder

“…Even with recent therapeutic advancements, such as the introduction of monoclonal antibody therapy such as rituximab, overall mortality rates can still be as high as 30-60% [9]. PTLD tends to have a better prognosis in the pediatric patient population compared with the adult population, with survival rates of up to 65-85% [8].…”

“…Overall, PTLD is the second most common malignancy in adult transplant recipients and the most common post-transplant malignancy in children [8]. Compared with de-novo lymphoma, PTLD may behave more aggressively, with patients experiencing poorer outcomes.…”

Incidental EBV-positivity in paediatric post-transplant specimens demonstrates the need for stringent criteria for diagnosing post-transplant lymphoproliferative disorders

“…As current clinical guidelines recommend reduction of immunosuppression and initiation of rituximab for patients with E/ND-PTLD, pathological distinction of E/ND-PTLD from non-PTLD lesions is required for proper therapeutic intervention 8 9…”

Rituximab

“…Severe cases need chemotherapy. Incorporation of rituximab in the treatment of PTLD has led to the decreased use of chemotherapy agents and better outcomes [25].…”