Rapidly progressive post-transplant lymphoproliferative disease following withdrawal of sirolimus

Abstract

Post-transplant lymphoproliferative disorder (PTLD) occurs in 1 -10% of patients undergoing solid-organ transplantation and presents predominantly in the first year. It is a heterogeneous entity and encompasses indolent B-or T-cell lymphoproliferation to aggressive monomorphic lymphoma. Sirolimus is an inhibitor of the response to interleukin-2 and blocks T-and B-cell activation. The drug has been used to treat renal transplant-related PTLD, but cases of PTLD related to stopping sirolimus have not been reported. We present a case of rapidly progressive central nervous system (CNS) PTLD 11 years after transplantation, in which sirolimus had been stopped 1 month prior to symptom onset. Case reportThe patient was a 31-year-old man who had undergone cadaveric renal transplantation 9 years previously for end-stage renal failure of undetermined cause. He presented with ataxia and acute confusion. In the 3 months preceding presentation he had experienced unexplained, asymptomatic weight loss.Clinical examination was unremarkable. Sirolimus had been stopped 1 month before the onset of symptoms, owing to an apparent functional iron deficiency in the absence of evident gastro-intestinal blood loss. The patient remained on mycophenolate mofetil and prednisone.On the morning of presentation, the patient's wife witnessed his confusion and unsteadiness. He was afebrile and normotensive,

Clinical characteristics and outcomes of PTLD following intestinal transplantation

“…32,33 Similarly, rapidly progressive central nervous system polymorphic EBV-related PTLD has been reported in a renal transplant recipient following withdrawal of sirolimus. 34 One of the most concerning findings of our study is the high cumulative incidence (43%) of second episodes of PTLD in our pediatric patients. Our experience represents the first report of second episodes of PTLD in a cohort of pediatric solid organ transplant recipients.…”

“…In addition, data suggest that sirolimus has potential anti-tumor properties. 34 One of the most concerning findings of our study is the high cumulative incidence (43%) of second episodes of PTLD in our pediatric patients. Clinically, mTOR inhibitors appear to have anti-proliferative properties and direct effect on carcinogenesis.…”

Primary central nervous system post-transplant lymphoproliferative disorders: the spectrum of imaging appearances and differential

“…There are no solid enhancing periventricular lesions as typically seen in CNS lymphoma. The location of lesions in the basal ganglia in cases c and d would be unusual for metastatic disease T2-weighted hypointensity related to hypercellularity but intermediate and increased T2-weighted signal intensities are also seen [16,[19][20][21]. The cystic and necrotic areas have signal intensities that follow fluid.…”

Primary central nervous system post-transplantation lymphoproliferative disorder: A case report and systematic review of imaging findings

“…6 A PRISMA flowchart of our screening and review process. [2 , 3 , [6] , [7] , [8] , [9] , [10] , [11] , [12] , [13] , [16] , [17] , [18] , [19] , [20] , [21] , [22] , [23] , [24] , [26] , [27] , [28] , [29] , [30] , [31] , [32] , [33] , [34] , [35] , [36] , 38 , [40] , [41] , [42] , [43] , [44] , [45] , [46] , [47] , [48] , [49] , [51] , [52] , [53] , [54] , 57 , [61] , [62] , [63] , [64] , [65] , [66] , [67] , [68] , [69] , [70] , [71] , [72] , [73] , [74] , …”