Successful treatment of central nervous system lymphoproliferative disorder in a kidney-pancreas and stem cell transplanted patient using intrathecal rituximab

Abstract

Central nervous system lymphoproliferative disorder (CNS-PTLD) after organ transplant is a unique clinicopathological entity and is associated with poor survival rates. When the CNS is involved, intravenous rituximab might not be the treatment of choice, due to its poor CNS penetration. However, intrathecal (IT) administration of rituximab has shown to be safe and efficient in small studies and in case series. We report here the case of a patient with late development of CNS-PTLD after kidney-pancreas transplantation who achieved complete remission after surgical resection and four cycles of IT rituximab and we provide a review of the literature for this treatment option.

Keywords: CNS cancer; haematology (drugs and medicines); haematology (incl blood transfusion); malignant disease and immunosuppression; neuroimaging.

Figure 1

Axial MRI spin echo T1 sequence after administration of gadolinium illustrates a right parietal lesion, well delineated, round, with a ring enhancement, surrounded by oedema.

Figure 2

Histology, immunohistochemistry and in situ hybridisation of the lesion. (A) H&E staining using standard techniques. (B) In situ hybridisation for the Epstein-Barr virus (EBV) -encoded RNA (EBER) transcript performed using the Ventana EBER1 800-2842 (750 ng/mL) (Ventana Medical Systems, Tucson, Arizona, USA) according to the manufacturer’s protocol. (C) CD20 staining using the antibody NCL-L-CD20-L26 from Novocastra (Leica Biosystems, Newcastle, UK). Staining was performed on a Ventana Benchmark ULTRA autostainer with antigen retrieval using the CC1 buffer for 120 min, incubation anti-CD20 (1/400) 60 min at 36°C followed by 3'3-diaminobenzidine HCl (DAB) revelation according to the manufacturer’s protocol. (D) The images show infiltration by large atypical cells positive for the B-cell marker CD20 and EBV EBER RNA. These cells were surrounded by necrosis that occupied >90% of the slide.