Case Presentation: A 25-year-old African American male with a history of Frasier syndrome, renal transplant, bilateral orchiectomy, and chronic immunosuppression presented to an outside hospital with a three-day history of shortness of breath and malaise. Workup revealed bilateral multifocal pneumonia and an acute kidney injury. He was transferred to the ICU at our hospital so he could be monitored by his transplant nephrology team. In addition to the patient’s acute problems, he was found to have a rash consisting of violaceous, hyperpigmented, or hypopigmented lesions with occasional overlying skin erosions as well as a large mass in the right inguinal region. A CT of the patient’s chest, abdomen, and pelvis revealed multiple loci of lymphadenopathy. Out of concern for post-transplant lymphoproliferative disorder, peripheral blood leukemia and lymphoma assays were performed. Biopsies of both the skin lesions and the inguinal mass were obtained, with the former showing epithelial hyperplasia and erosions consistent with prurigo nodularis. The biopsy of the inguinal mass and the peripheral blood markers both revealed the presence of CD4+ T-cell lymphoma cells. Upon this diagnosis, the patient was transferred to our oncology team for treatment.
Discussion: Post-transplant lymphoproliferative disorder (PTLD) is a serious complication of renal transplantation. PTLD is most often a proliferation of B cells related to reactivation of Epstein-Barr virus (EBV) and can be benign or malignant. Diffuse large B-cell lymphoma and Burkitt lymphoma are the most common malignant PTLD subtypes.(1) Here, we present a rare case of PTLD that presents as not a B-cell lymphoma but as a T-cell lymphoma in an EBV seronegative patient. We present this case of T-cell lymphoma to bring awareness and inform clinical practice for this rare and challenging case that requires multidisciplinary care. Current literature on prevention of PTLD focuses on antiviral prophylaxis and prompt treatment of viral reactivation. However, such preventative measures fall short when it comes to identifying how to prevent PTLD in the minority of patients who are EBV seronegative and have T-cell derived PTLD. A key component of PTLD treatment is reduction of immunosuppression and, similarly, evidence suggests that aggressive tapering of immunosuppression is another means for PTLD prevention.(2) Input from several medical teams can be needed to weigh options between treating the PTLD with reduced immunosuppression or using other treatments in order to spare the transplanted organ from possible graft rejection.
Conclusions: T-cell lymphoma is a rare manifestation of PTLD and requires multidisciplinary care. We present this case to add to the limited literature related to this topic and also increase awareness among practitioners to consider this diagnosis. More research is needed to understand the diagnosis and treatment.