TY - JOUR
T1 - Primary CNS posttransplant lymphoproliferative disease (PTLD)
T2 - An international report of 84 cases in the modern era
AU - Evens, A. M.
AU - Choquet, S.
AU - Kroll-Desrosiers, A. R.
AU - Jagadeesh, D.
AU - Smith, S. M.
AU - Morschhauser, F.
AU - Leblond, V.
AU - Roy, R.
AU - Barton, B.
AU - Gordon, L. I.
AU - Gandhi, M. K.
AU - Dierickx, D.
AU - Schiff, D.
AU - Habermann, T. M.
AU - Trappe, R.
PY - 2013/6
Y1 - 2013/6
N2 - We performed a multicenter, International analysis of solid organ transplant (SOT)-related primary central nervous system (PCNS) posttransplant lymphoproliferative disease (PTLD). Among 84 PCNS PTLD patients, median time of SOT-to-PTLD was 54 months, 79% had kidney SOT, histology was monomorphic in 83% and tumor was EBV+ in 94%. Further, 33% had deep brain involvement, 10% had CSF involvement, while none had ocular disease. Immunosuppression was reduced in 93%; additional first-line therapy included high-dose methotrexate (48%), high-dose cytarabine (33%), brain radiation (24%) and/or rituximab (44%). The overall response rate was 60%, while treatment-related mortality was 13%. With 42-month median follow-up, three-year progression-free survival (PFS) and overall survival (OS) were 32% and 43%, respectively. There was a trend on univariable analysis for improved PFS for patients who received rituximab and/or high-dose cytarabine. On multivariable Cox regression, poor performance status predicted inferior PFS (HR 2.61, 95% CI 1.32-5.17, p = 0.006), while increased LDH portended inferior OS (HR 4.16, 95% CI 1.29-13.46, p = 0.02). Moreover, lack of response to first-line therapy was the most dominant prognostic factor on multivariable analysis (HR 8.70, 95% CI 2.56-29.57, p = 0.0005). Altogether, PCNS PTLD appears to represent a distinct clinicopathologic entity within the PTLD spectrum that is associated with renal SOT, occurs late, is monomorphic and retains EBV positivity. In an international analysis of solid organ transplant recipients with primary central nervous system posttransplant lymphoproliferative disease, the authors find that the disease is associated with renal transplantation, typically occurs late and retains EBV positivity, and the dominant prognostic factor for survival is response to first-line therapy.
AB - We performed a multicenter, International analysis of solid organ transplant (SOT)-related primary central nervous system (PCNS) posttransplant lymphoproliferative disease (PTLD). Among 84 PCNS PTLD patients, median time of SOT-to-PTLD was 54 months, 79% had kidney SOT, histology was monomorphic in 83% and tumor was EBV+ in 94%. Further, 33% had deep brain involvement, 10% had CSF involvement, while none had ocular disease. Immunosuppression was reduced in 93%; additional first-line therapy included high-dose methotrexate (48%), high-dose cytarabine (33%), brain radiation (24%) and/or rituximab (44%). The overall response rate was 60%, while treatment-related mortality was 13%. With 42-month median follow-up, three-year progression-free survival (PFS) and overall survival (OS) were 32% and 43%, respectively. There was a trend on univariable analysis for improved PFS for patients who received rituximab and/or high-dose cytarabine. On multivariable Cox regression, poor performance status predicted inferior PFS (HR 2.61, 95% CI 1.32-5.17, p = 0.006), while increased LDH portended inferior OS (HR 4.16, 95% CI 1.29-13.46, p = 0.02). Moreover, lack of response to first-line therapy was the most dominant prognostic factor on multivariable analysis (HR 8.70, 95% CI 2.56-29.57, p = 0.0005). Altogether, PCNS PTLD appears to represent a distinct clinicopathologic entity within the PTLD spectrum that is associated with renal SOT, occurs late, is monomorphic and retains EBV positivity. In an international analysis of solid organ transplant recipients with primary central nervous system posttransplant lymphoproliferative disease, the authors find that the disease is associated with renal transplantation, typically occurs late and retains EBV positivity, and the dominant prognostic factor for survival is response to first-line therapy.
KW - CNS
KW - PTLD
KW - lymphoma
KW - prognosis
KW - rituximab
UR - http://www.scopus.com/inward/record.url?scp=84878517158&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84878517158&partnerID=8YFLogxK
U2 - 10.1111/ajt.12211
DO - 10.1111/ajt.12211
M3 - Article
C2 - 23721553
AN - SCOPUS:84878517158
SN - 1600-6135
VL - 13
SP - 1512
EP - 1522
JO - American Journal of Transplantation
JF - American Journal of Transplantation
IS - 6
ER -