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Abstract: PO2467

Post-Transplant Lymphoproliferative Disorder: Recurrence at an Unusual Site

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
  • Conde, Miguel, Saint Barnabas Medical Center, Livingston, New Jersey, United States
Introduction

PTLD includes a spectrum of clinical presentations due to lymphoid proliferation ranging from benign hyperplasia to aggressive lymphomas that occur after either a SOT or HSCT. PTLD can involve extra nodal sites like graft tissue, GI tract, and lungs. Skin and soft tissue involvement is very rare. We report a patient with PTLD who had relapsed with axillary lymphadenopathy and a soft tissue mass on the arm.

Case Description

A 59-year-old male was diagnosed with EBV negative PTLD involving retroperitoneal, mediastinal and supraclavicular lymph node, one year after renal transplant. He was treated R-CEOP (Rituximab, Cyclophosphamide, Etoposide, Vincristine, Prednisone). After 6 cycles of chemotherapy repeat PET scan confirmed complete resolution. 3 months later, he presented with LUE pain and swelling. Labs were significant for high LDH and Beta-2 microglobulin. CT LUE revealed 4.8 cm mass over left anterior arm and left axillary adenopathy. Biopsy and flow cytometry of axillary lymph node and left arm mass confirmed relapse. Immunohistochemical stains showed tumor cells positive for CD20, PAX5, BCL2, MUM1, BCL6 with Ki67 of 80-90%. Patient developed compartment syndrome and had to undergo fasciotomy. Patient was started on modified regimen with Rituximab, Gemcitabine and Oxaliplatin (R-GEMOX) for poor performance status.

Discussion

The incidence of PTLD ranges from 1 to 25% with 90% of cases being EBV-associated, CD 20 positive, B cell neoplasms. EBV negative PTLD occurs only in 5-10% cases, appears late with worse prognosis than EBV positive PTLD. EBV negative PTLD is assumed to be related to Tp53 mutation caused by immunosuppressive agents like azathioprine or tacrolimus. Skin and subcutaneous lesions are extremely rare as sites of extra nodal presentation and may take the form of solitary or multiple papules, nodules, plaques with ulceration, comedo-like lesions, follicular keratotic papules, or localized alopecia.Our patient is interesting as he had a relapse of EBV negative PTLD in the form of a soft tissue fibro-adipose mass in the upper extremity. To the best of our knowledge, there have been three cases of PTLD presenting as soft tissue masses on head reported in the literature. Biopsy remains the gold standard for diagnosis. Treatment is includes reduction of immunosuppression and rituximab with additional chemotherapy.