A Rare Presentation of Isolated CNS Posttransplantation Lymphoproliferative Disorder

Abstract

Posttransplantation lymphoproliferative disorder (PTLD) is a recognized and extremely morbid complication of solid organ transplantation, but central nervous system involvement, particularly in isolation, is rare. There are no standardized treatment strategies for PTLD, though commonly used strategies include reduction of immunosuppression, chemotherapy, rituximab, radiation, and surgery. We present a case of an unusual morphologic variant of primary central nervous system PTLD with successful response to rituximab and cranial radiation. A 69-year-old Asian male, who underwent postrenal transplant nine years earlier, presented with a one-month history of new onset seizure activity. His evaluation revealed multiple brain lesions on magnetic resonance imaging (MRI), as well as serologic and cerebrospinal fluid studies which were positive for Epstein-Barr Virus (EBV) infection. Ultimately, he underwent craniotomy with tissue biopsy with the final pathology report showing posttransplant lymphoproliferative disorder, polymorphic type. The patient was managed with reduction in immunosuppression, rituximab therapy, and cranial radiation treatments. He had demonstrated marked improvement in his neurologic function and was ultimately discharged to inpatient rehabilitation facility.

The clinical impact of human T-lymphotrophic virus type 1 (HTLV-1) infection on the development of adult T-cell leukemia-lymphoma (ATL) or HTLV-1–associated myelopathy (HAM) / atypical HAM after allogeneic hematopoietic stem cell transplantation (allo-HSCT) and renal transplantation

“…One HTLV-1 non-carrier in our study developed PTLD (EBV+) in the brain after renal transplantation instead of ATL. Immediate diagnosis and subsequent treatment, including rituximab plus HDAC or HD-MTX, did not improve PTLD in the brain according to a review by Nagel et al ., 37 a previous report of PTLD in the brain, 15 , 38 and previous reports on PTLD treatments 39 - 46 . Thus, careful follow-up may also be necessary to detect the development of PTLD after renal transplantation.…”

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

“…ITSS within intracranial lesions is thought to be the result of tumoral microhemorrhage, necrosis, and angiogenesis. A few cases of significant hemorrhage causing acute neurological symptoms, attributed to PCNS-PTLD, have been reported [20 , 51] . Hemorrhage and necrosis are known to increase following treatment, and increased ITSS is expected ( Fig.…”

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Spontaneous regression of central nervous system posttransplant lymphoproliferative disease

“…[ 7 ] Morris J, et al reported a patient treated with RI, rituximab and cranial radiation. [ 8 ] Arita H, et al presented two cases of P-PTLD treated with RI plus steroids and surgery plus irradiation separately. [ 9 ] Velvet AJJ, et al reported a case treated with RI, craniotomy, radiotherapy and rituximab.…”