UMass Chan Medical School Faculty Publications


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


Document Type



Lymphoproliferative Disorders; Organ Transplantation


Amino Acids, Peptides, and Proteins | Hematology | Hemic and Lymphatic Diseases | Immune System Diseases | Neoplasms | Nervous System | Oncology | Pharmaceutical Preparations | Surgery | Surgical Procedures, Operative | Therapeutics


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


CNS, lymphoma, prognosis, PTLD, rituximab

DOI of Published Version



Am J Transplant. 2013 Apr 3. doi: 10.1002/ajt.12211. Link to article on publisher's site

Related Resources

Link to Article in PubMed

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