Primary CNS posttransplant lymphoproliferative disease (PTLD): An international report of 84 cases in the modern era

Evens A.M., Choquet S., Kroll-Desrosiers A.R., Jagadeesh D., Smith S.M., Morschhauser F., Leblond V., Roy R., Barton B., Gordon L.I., Gandhi M.K., Dierickx D., Schiff D., Habermann T.M. and Trappe R. (2013) Primary CNS posttransplant lymphoproliferative disease (PTLD): An international report of 84 cases in the modern era. American Journal of Transplantation, 13 6: 1512-1522. doi:10.1111/ajt.12211

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Author Evens A.M.
Choquet S.
Kroll-Desrosiers A.R.
Jagadeesh D.
Smith S.M.
Morschhauser F.
Leblond V.
Roy R.
Barton B.
Gordon L.I.
Gandhi M.K.
Dierickx D.
Schiff D.
Habermann T.M.
Trappe R.
Title Primary CNS posttransplant lymphoproliferative disease (PTLD): An international report of 84 cases in the modern era
Journal name American Journal of Transplantation   Check publisher's open access policy
ISSN 1600-6135
Publication date 2013
Sub-type Article (original research)
DOI 10.1111/ajt.12211
Open Access Status
Volume 13
Issue 6
Start page 1512
End page 1522
Total pages 11
Place of publication Malden, MA, U.S.A.
Publisher Wiley-Blackwell Publishing, Inc.
Collection year 2014
Language eng
Subject 2747 Transplantation
2723 Immunology and Allergy
2736 Pharmacology (medical)
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. In an international analysis of solid organ transplant recipients with primary central nervous system posttransplant lymphoproliferative disease, the authors find that the disease is associated with renal transplantation, typically occurs late and retains EBV positivity, and the dominant prognostic factor for survival is response to first-line therapy.
Keyword CNS
Q-Index Code C1
Q-Index Status Confirmed Code
Institutional Status UQ

Document type: Journal Article
Sub-type: Article (original research)
Collections: Official 2014 Collection
School of Medicine Publications
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Created: Mon, 19 May 2014, 10:43:48 EST by Matthew Lamb on behalf of School of Medicine