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Abstract: FR-PO671

Unique Presentations of Post-Renal Transplant Gamma Delta T Cell Lymphoma

Session Information

Category: Trainee Case Report

  • 1500 Onco-Nephrology

Authors

  • Patil, Rujuta R., Wayne State University School of Medicine, Detroit, Michigan, United States
  • Patel, Anita K., Henry Ford Hospital, West Bloomfield, Michigan, United States
Introduction

Post-transplant lymphoproliferative disorder (PTLD) is serious complication occurring in up to 10% of solid organ transplant recipients (R). In monomorphic PTLD, the majority of cases arise from B cells (good prognosis) and rarely from T cells. Less than 5% of T-cell lymphomas express gamma delta T-cell receptors. Gamma delta T cell lymphomas (GDTL) are outlined into two groups: hepatosplenic (HSGDTL) and primary cutaneous (PCGDTL). We present 2 recipients that developed PCGDTL and HSGDTL.

Case Description

R1 was a 67-year-old male who received a living, unrelated renal transplant, induced with Simulect. He was EBV IgG + and mismatched CMV IgG -. He presented 1-year post transplant with 20lb weight loss, pruritic skin rash, no EBV DNAemia, and diffuse lymphadenopathy on body PET CT. Biopsy of left axillary lymph node, skin, and bone marrow revealed a mature stage 4 T-cell lymphoma (CD2+, CD3+, CD4+, CD5+, CD7+, CD8-, CD45+, TCR gamma-delta TCR+, and alpha-beta TCR-). This case of PCGDTL had an unusual phenotype of CD4 positivity with presentation in lymph nodes and bone marrow. Immunosuppression was discontinued. Following treatment with 6 cycles of EPOCH regimen with complete resolution, his disease relapsed 2 months later and he died with a functioning allograft within 1 year of diagnosis.

R2 was a 44-year-old male who received a deceased donor renal transplant, induced with Thymoglobulin. He was EBV IgG+ and non-mismatched CMV IgG-. He presented 7 years post transplant with abdominal pain, abnormal liver function tests, no EBV DNAemia, and moderate hepatosplenomegaly on abdominal CT. Peripheral blood flow cytometry and bone marrow biopsy revealed HSGDTL (CD2+, CD3+, CD4-, CD5-, CD7+, CD8 (dimly positive), CD25-, gamma/delta TCR+, and alpha/beta TCR-). Immunosuppression was discontinued. He was treated with 2 cycles of CHOEP regimen with treatment failure and 3 cycles of salvage therapy (Gemcitabine, Decadron, Carboplatin) with complete resolution. He relapsed with leukemic conversion 2 months later and died with a functioning allograft.

Discussion

Our cases demonstrate that GDTL is an aggressive neoplasia with rapid onset and poor prognosis. Despite initial response, both recipients died within 1 year of diagnosis. PCGDTL seems to clinically present earlier than HSGDTL. Also, neither of our patients had CMV/EBV viremia, suggesting GDTL development is potentially independent of virology.