alexa Primary CNS posttransplant lymphoproliferative disease (PTLD): an international report of 84 cases in the modern era.
Infectious Diseases

Infectious Diseases

Journal of Infectious Diseases & Therapy

Author(s): Evens AM, Choquet S, KrollDesrosiers AR, Jagadeesh D, Smith SM,

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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. © Copyright 2013 The American Society of Transplantation and the American Society of Transplant Surgeons. This article was published in Am J Transplant and referenced in Journal of Infectious Diseases & Therapy

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