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Case Reports

Abstract

Post-transplant lymphoproliferative disorders (PTLDs) are mostly associated with Epstein-Barr virus (EBV) and are usually monomorphic B-cell type. EBV-negative PTLDs are unusual and mostly occur after a median of 50 months post-transplantation. We describe the case of an 11-year-old female with a history of multi-visceral organ transplant including liver, pancreas, gut, and kidney due to short gut syndrome, who presented with 20 days of diffuse mildly pruritic papular rash also involving the face. The rash started 34 days after transplantation. The rash was unresponsive to prednisone and triamcinolone, and the clinical considerations included GVHD and drug eruption. A skin biopsy from the left mid back demonstrated dermal infiltrate composed of mononuclear cells with cytologic atypia, irregular and folded nuclei and moderate amounts of cytoplasm.  Scattered eosinophils were also seen. Immunohistochemistry demonstrated that the atypical cells were positive for CD3 and CD5, with a subset positive for CD4. Some atypical cells expressed CD8, TIA-1, and granzyme B. Aberrant partial loss of CD7 immunoexpression was noted, and the Ki-67 proliferation rate was high. The atypical cells were negative for CD20, CD30, ALK, TdT, CD34, CD1a, MPO, and CD123. EBER ISH was negative. Findings were consistent with EBV-negative T-cell PTLD. Subsequently, small bowel biopsies revealed EBV-positive diffuse large B-cell lymphoma. The patient completed 6 cycles of chemotherapy and is in complete remission. EBV-negative T-cell PTLD is rare, especially in such a short time after transplant, and its occurrence in conjunction with a monomorphic B-cell PTLD is unusual.