Effective management of primary central nervous system posttransplant lymphoproliferative disorder in a kidney transplant recipient using surgery and rituximab, along with a literature review

Background: Primary central nervous system post-transplant lymphoproliferative disorder (PCNS-PTLD) is a rare but severe complication following solid organ transplantation (SOT). Currently, treatment regimens still lack clear guidelines. Methods: A kidney transplant recipient with PCNS-PTLD was reported in this case study, who treated with rituximab after transplant surgery. What's more, PubMed was used to find case series related to PCNS-PTLD. Results: The patient of this case report experienced complete remission (CR) following resection and treatment with rituximab. A total of 130 cases were extracted from 20 articles and were combined with one case from our institution. Out of 131 patients with PCNS-PTLD, the median duration between SOT and PTLD was 48 months. The majority (83 %) of patients had received a kidney transplant, with 74.8 % showing monomorphic histology and 93 % having an EBV+ tumor. Most patients (95 %) had reduction in immunosuppression as part of their first- line treatment. Other initial treatments consisted of high-dose methotrexate (HD-MTX) (46 %), high-dose cytarabine (HDAC) (26 %), and/or rituximab (47 %). The Overall Response Rate (ORR) was 63 %, showing that HD-MTX and/or HDAC-based therapy had the highest rates of ORR and CR. Roughly half of the participants experienced prolonged survival. After 36 months of observation, the median progression free survival (PFS) was 10 months and the overall survival (OS) was 18 months. Conclusion: The use of HD-MTX and HDAC showed promise in treating PCNS-PTLD, but rituximab may also a potential drug for the PCNS-PTLD. Research should continue to investigate the alternative treatments for this condition.