TY - JOUR
T1 - Intraventricular rituximab and systemic chemotherapy for treatment of central nervous system post-transplant lymphoproliferative disorder after kidney transplantation
AU - Twombley, Katherine
AU - Pokala, Hanumantha
AU - Ardura, Monica I.
AU - Harker-Murray, Paul
AU - Johnson-Welch, Sarah F.
AU - Weinberg, Arthur
AU - Seikaly, Mouin
PY - 2012/9
Y1 - 2012/9
N2 - PTLD of the CNS is a rare complication of solid organ transplantation, and there are only case reports/series available in the literature. Current literature suggests that CNS PTLD carries a worse prognosis than PTLD outside the CNS, and most are of B-cell lineage, predominately monomorphic, and are associated with EBV infection. Because this disorder is so rare, there is no standard chemotherapy for pediatric patients with CNS PTLD and reported therapies for EBV-associated CNS PTLD are heterogeneous with mixed results. Since outcomes of CNS PTLD are historically poor, we attempted to develop a novel therapeutic treatment regimen. Based on a review of the literature and with the help of a multidisciplinary team, we created a regimen of chemotherapy that included dexamethasone and high-dose methotrexate in addition to intravenous and intraventricular Rituximab in two pediatric patients. The intraventricular chemotherapy succeeded in shrinking the tumor in both of our patients; however, as shown in the second case, the clinical outcome depends on the location of the tumor. Systemic and intraventricular therapies hold promise in the management of EBV-associated CNS PTLD; however the rarity of this entity prevents the development of well-designed studies necessary for the establishment of an evidence-based treatment standard.
AB - PTLD of the CNS is a rare complication of solid organ transplantation, and there are only case reports/series available in the literature. Current literature suggests that CNS PTLD carries a worse prognosis than PTLD outside the CNS, and most are of B-cell lineage, predominately monomorphic, and are associated with EBV infection. Because this disorder is so rare, there is no standard chemotherapy for pediatric patients with CNS PTLD and reported therapies for EBV-associated CNS PTLD are heterogeneous with mixed results. Since outcomes of CNS PTLD are historically poor, we attempted to develop a novel therapeutic treatment regimen. Based on a review of the literature and with the help of a multidisciplinary team, we created a regimen of chemotherapy that included dexamethasone and high-dose methotrexate in addition to intravenous and intraventricular Rituximab in two pediatric patients. The intraventricular chemotherapy succeeded in shrinking the tumor in both of our patients; however, as shown in the second case, the clinical outcome depends on the location of the tumor. Systemic and intraventricular therapies hold promise in the management of EBV-associated CNS PTLD; however the rarity of this entity prevents the development of well-designed studies necessary for the establishment of an evidence-based treatment standard.
KW - Epstein-Barr virus
KW - pediatric kidney transplant
KW - post-transplant lymphoproliferative
KW - post-transplant lymphoproliferative disorder
KW - rituximab
UR - http://www.scopus.com/inward/record.url?scp=84864609976&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84864609976&partnerID=8YFLogxK
U2 - 10.1111/j.1399-3046.2012.01699.x
DO - 10.1111/j.1399-3046.2012.01699.x
M3 - Article
C2 - 22646132
AN - SCOPUS:84864609976
SN - 1397-3142
VL - 16
SP - E201-E209
JO - Pediatric Transplantation
JF - Pediatric Transplantation
IS - 6
ER -