title: Primary CNS posttransplant lymphoproliferative disease (PTLD): an international report of 84 cases in the modern era.
abstract: 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.
authors.affiliation: Division of Hematology/Oncology, The University of Massachusetts Medical School, Worcester, MA, USA. andrew.evens@umassmed.edu
authors.forename: A M
authors.initials: AM
authors.lastname: Evens
authors.forename: S
authors.initials: S
authors.lastname: Choquet
authors.forename: A R
authors.initials: AR
authors.lastname: Kroll-Desrosiers
authors.forename: D
authors.initials: D
authors.lastname: Jagadeesh
authors.forename: S M
authors.initials: SM
authors.lastname: Smith
authors.forename: F
authors.initials: F
authors.lastname: Morschhauser
authors.forename: V
authors.initials: V
authors.lastname: Leblond
authors.forename: R
authors.initials: R
authors.lastname: Roy
authors.forename: B
authors.initials: B
authors.lastname: Barton
authors.forename: L I
authors.initials: LI
authors.lastname: Gordon
authors.forename: M K
authors.initials: MK
authors.lastname: Gandhi
authors.forename: D
authors.initials: D
authors.lastname: Dierickx
authors.forename: D
authors.initials: D
authors.lastname: Schiff
authors.forename: T M
authors.initials: TM
authors.lastname: Habermann
authors.forename: R
authors.initials: R
authors.lastname: Trappe
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002493:Central Nervous System Diseases / Q000453:epidemiology* / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D014943:Global Health; D006801:Humans; D015994:Incidence; D008232:Lymphoproliferative Disorders / Q000453:epidemiology* / Q000209:etiology; D008297:Male; D008875:Middle Aged; D016377:Organ Transplantation / Q000009:adverse effects*; D011183:Postoperative Complications; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D055815:Young Adult