Primary CNS Posttransplant Lymphoproliferative Disease (PTLD): An International Report of 84 Cases in the Modern Era
Department of Quantitative Health Sciences; Department of Medicine, Division of Hematology/Oncology
Medical Subject Headings
Lymphoproliferative Disorders; Organ Transplantation
Hematology | Hemic and Lymphatic Diseases | Oncology | Surgery
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.
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Citation: Am J Transplant. 2013 Apr 3. doi: 10.1002/ajt.12211. Link to article on publisher's site
Evens, Andrew M.; Choquet, S.; Kroll-Desrosiers, Aimee R.; Jagadeesh, Deepa; Smith, S. M.; Morschhauser, F.; Leblond, V.; Roy, R.; Barton, Bruce A.; Gordon, L. I.; Gandhi, M. K.; Dierickx, D.; Schiff, D.; Habermann, T. M.; and Trappe, R., "Primary CNS Posttransplant Lymphoproliferative Disease (PTLD): An International Report of 84 Cases in the Modern Era" (2013). University of Massachusetts Medical School Faculty Publications. 101.