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Abstract: PO2468

Post-Transplant Lymphoproliferative Disorder (PTLD) Presenting as Solitary CNS Lymphoma: A Rare Occurrence

Session Information

Category: Trainee Case Report

  • 1902 Transplantation: Clinical

Authors

  • Saha, Aditi, Saint Barnabas Medical Center, Livingston, New Jersey, United States
  • Tariq, Sohaib, Saint Barnabas Medical Center, Livingston, New Jersey, United States
  • Thirunavukarasu Murugan, Lalithaa, Saint Barnabas Medical Center, Livingston, New Jersey, United States
  • Sapru, Sunil, Saint Barnabas Medical Center, Livingston, New Jersey, United States
  • Grossman, Israel Robert, Saint Barnabas Medical Center, Livingston, New Jersey, United States
Introduction

PTLD associated lymphoma is the second most common malignancy in patients receiving SOT or HSCT with an incidence rate of 1%-3%. PCNS-PTLD occurs in 7%-15% of all PTLD cases. We present a case of isolated PCNS lymphoma two years after renal transplant.

Case Description

A 57 year old woman with PMH of ESRD s/p kidney transplant (D/EBV -,R/ EBV +) with chronic CrCl of 30-40% presented with paresthesia and numbness over the right side of her body with mild ataxia 2 years post transplant. Physical exam revealed only numbness over the right side of body. MRI with contrast revealed a left thalamic lesion with moderate vasogenic edema, and MR spectroscopy confirmed high grade neoplasm involving left thalamus. Stereotactic biopsy showed polymorphic CD-20 and EBV positive PTLD. EBV PCR were elevated. Further evaluation ruled out systemic PTLD. Myfortic was stopped and oral steroids started with mild improvement in symptoms. Choice of systemic chemotherapy was limited due to reduced CrCl and risks of graft failure. She received modified regimen with renally adjusted high dose Methotrexate, Vincristine and Rituximab for 6 cycles with partial remission and then Temozolomide for 7 cycles with complete remission.

Discussion

The incidence of PTLD ranges from 1 to 25% with 90% of cases being EBV-associated, CD20 positive, B cell neoplasms. PCNS-PTLD has a higher incidence in renal SOT, occurs late and is usually monomorphic unlike our patient. Median time of occurrence is 4-5 years after transplant. Risk factors include age, intensity of immunosuppression, time to transplant and EBV status of donor and recipient. Common presenting features include neurological deficits, seizure, and raised intracranial pressure. MRI is the preferred imaging and shows multifocal, ill defined, ring enhancing lesions usually in supratentorial and lobar regions. Positive CSF EBV PCR is highly suggestive but biopsy remains the gold standard for diagnosis. Treatment modalities include reduction of immunosuppression, rituximab, high dose methotrexate, cytarabine and cranial radiotherapy. Use of high dose methotrexate has shown improved outcomes with median survival of 26-47 months. Lack of response to first line therapy is considered the dominant prognostic factor. Early recognition and diagnosis remain crucial for improving outcome.