Post-transplantation lymphoproliferative disorders: Current concepts and future therapeutic approaches

Table 3 Early vs late onset post-transplant lymphoproliferative disorders in pediatrics[46]

	Early PTLD	Late PTLD
General criteria	Diffuse large B-cell or other B-cell lymphoma	Burkitt’s lymphoma and Hodgkin’s disease are late events[47]
	Atypical presentation (graft dysfunction, abdominal pain, frequent extra-nodal involvement in > 80% of TR)[46]	Frequent EBV negative tumors. Specific tumorigenic events e.g., C-myc translocations are restricted to late PTLDs
Time to PTLD	Shortest for lung, heart/lung TR. Early PTLD is quite frequent in liver TR (Late PTLD beyond 5 yr is rare, immunosuppression can be tapered/hold due to tolerance)	Longest for the heart TR and at risk for late PTLD even > 10 yr after trans-plantation
Patient survival	No significant difference in most published studies[20,47-49]
Distinct criteria	B-cell origin, almost exclusively EBV+ve, reflecting reduced immunosurv-eillance as major pathogenetic factor	Resembles tumors with distinct pathogenetic alterations and nodal appearance[46]
Role of immunos-uppression	Induction therapy has a role. More likely to develop graft rejection and switch to Tac before PTLD diagnosis	Cumulative immunosuppression is crucial

Tx: Transplantation; TR: Transplant recipient; PTLD: Post-transplant lymphoproliferative disorders; EBV: Epstein-Barr virus; HSCT: Haplo-identical allogeneic hematopoietic stem-cell transplant; LDH: Lactate dehydrogenase.