UMass Chan Medical School Faculty Publications
Title
Primary CNS Posttransplant Lymphoproliferative Disease (PTLD): An International Report of 84 Cases in the Modern Era
UMMS Affiliation
Department of Quantitative Health Sciences; Department of Medicine, Division of Hematology/Oncology
Publication Date
2013-04-03
Document Type
Article
Subjects
Lymphoproliferative Disorders; Organ Transplantation
Disciplines
Amino Acids, Peptides, and Proteins | Hematology | Hemic and Lymphatic Diseases | Immune System Diseases | Neoplasms | Nervous System | Oncology | Pharmaceutical Preparations | Surgery | Surgical Procedures, Operative | Therapeutics
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
Keywords
CNS, lymphoma, prognosis, PTLD, rituximab
DOI of Published Version
10.1002/ajt.12211
Source
Am J Transplant. 2013 Apr 3. doi: 10.1002/ajt.12211. Link to article on publisher's site
Related Resources
Journal/Book/Conference Title
American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons
PubMed ID
23551686
Repository Citation
Evens AM, Choquet S, Kroll-Desrosiers AR, Jagadeesh D, Smith SM, Morschhauser F, Leblond V, Roy R, Barton BA, Gordon LI, Gandhi MK, Dierickx D, Schiff D, Habermann TM, Trappe R. (2013). Primary CNS Posttransplant Lymphoproliferative Disease (PTLD): An International Report of 84 Cases in the Modern Era. UMass Chan Medical School Faculty Publications. https://doi.org/10.1002/ajt.12211. Retrieved from https://escholarship.umassmed.edu/faculty_pubs/101