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Abstract: TH-PO841

Triple Jeopardy: Trialled Thrice in the Same Crime

Session Information

Category: Transplantation

  • 2102 Transplantation: Clinical


  • Ayub, Fatima, University of Arkansas for Medical Sciences, Little Rock, Arkansas, United States
  • Hasan, Md Rajibul, Arkansas College of Osteopathic Medicine, Fort Smith, Arkansas, United States
  • Khan, Nasir, University of Arkansas for Medical Sciences, Little Rock, Arkansas, United States
  • Bin Homam, Wadhah Mohammed, University of Arkansas for Medical Sciences, Little Rock, Arkansas, United States
  • Manchala, Venkata Ramana, University of Arkansas for Medical Sciences, Little Rock, Arkansas, United States

Post-transplant lymphoproliferative disorder (PTLD) is a life threatening complication following solid organ transplantation. PTLD covers a wide range of lymphomas, however the most common is the B-cell type. While majority of the cases are associated with Epstein-Barr virus (EBV) infection, about 20 % of the PTLD can occur in EBV seronegative patients. Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive immune activation that can rapidly progress to multisystem organ failure and is mostly associated with infections and malignancies. Only two cases of HLH associated with EBV negative PTLD in a renal transplant patient have been reported in the literature. Hereby, we present an extremely rare (third) case of EBV-negative PTLD complicated by HLH in a kidney transplant recipient.

Case Description

41 year old male with history of deceased donor kidney transplant for hypertensive nephrosclerosis about 10 years ago presented to the emergency department with complains of fever, fatigue and diarrhea for 3 weeks. His maintenance immunosuppression included tacrolimus, prednisone and mycophenolate. On presentation, he was hypotensive and febrile. Initial labs showed leukocytosis, anemia, thrombocytopenia, acute kidney injury, hypoglycemia, and severe lactic acidosis. The patient was started on broad spectrum antibiotics. Further work up revealed an undetected plasma EBV. His tremendously high serum ferritin, lactate dehydrogenase, triglycerides, soluble IL-2 receptor levels and abdominal imaging revealing splenomegaly did raise concern for HLH secondary to an underlying malignancy. He underwent bone marrow biopsy which showed findings consistent with diffuse large B cell lymphoma (DLBCL). He subsequently received 6 cycles of chemotherapy with R-CHOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone) regimen resulting in complete remission of DLBCL.


The incidence of PTLD in adult kidney transplant recipients ranges from 1-3%. The principal risk factors for PTLD include degree of immunosuppression and the EBV serologic status of the recipient. Clinical manifestations are non-specific and highly variable. B cell lymphoma with HLH in kidney transplant recipients is a rare entity and associated with a high mortality of >50%. Therefore, a high index of clinical suspicion and prompt initiation of chemotherapy is the key.