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Post‐transplantation lymphoproliferative disease in pediatric kidney transplant recipients—early success does not mean the battle is over!

Post-transplantation lymphoproliferative disease (PTLD) is one of the most serious complications of kidney transplant (KT) and entails the vast majority (86%) of malignancies of pediatric KT recipients. The estimated 5-year incidence in this population is 1–2% [1–3]. PTLD results from the necessary post-transplantation immunosuppression (IS) protocol that targets prevention of graft rejection but impairs defense against certain infectious triggers, specifically EBV, for which pediatric KT recipients are mostly immunologically na€ıve [2–4]. Significantly malignancy is responsible for 11.5–15% of all mortality cases in this young population [1,2,4,5]. Kanzelmeyer et al. [6] described their center’s experience with pediatric KT recipients followed for 3 years after PTLD diagnosis. Their goal was to evaluate their structured PTLD protocol: Ped-PTLD-2005, which was previously described [2]. In the current report, PTLD therapy, consisted mainly of CNI withdrawal (in 10/12 patients), mTOR introduction (in 9/12) and rituximab administration (9/12). Chemotherapy was administered to only two of 12 patients of their cohort: one with non-EBV-PTLD and another with Hodgkin lymphoma. The main outcomes examined were PTLD course and graft survival during at least 3 years follow-up. The results reported are remarkable, with 100% patient survival. This exceeds the considerably lower survival rates of 64–83% [2–5,7], and even the recently reported 87.4% survival rate after PTLD diagnosis [8]. The high rate of patient survival in Kanzelmeyer et al.’s cohort [6] is even more noteworthy in light of the diagnosis of the high grade monomorphic PTLD, which was detected in more than half of their patients (7/12). This PTLD type is less frequently reported in pediatric patients and has a worse prognosis [4,7,9]. However, the EBV-driven PTLD type (found in 11/12 of the presented cohort) and the early onset of malignancy (first post-transplantation year in 7/12) are known to associate with better prognosis [3,4,10], as is the timely introduction of Rituximab. The latter likely has an important role in modulation of this mainly B-cell dysregulation-induced tumor [2–4]. Also of note is the high rate of PTLD remission achieved with the adopted protocol in Kanzelmeyer et al.’s cohort. The high rate of graft preservation observed in 10 of the 12 patients in the described cohort, during at least 3 years post-PTLD diagnosis and treatment [6], is also of note. The rate was actually 100%, as during this period, the three graft losses were due to the basal disease: two aHUS at a time when eculizumab was not available and