Fatal Case of Ebv-Negative Posttransplant Lymphoproliferative Disorder With Hemophagocytic Lymphohistiocytosis in an Adult Kidney Transplant Recipient

Authors

Sergio Mazzola Poli de Figueiredo
John C Johnson
Kirill A Lyapichev
Heather L Stevenson
Alfred Lea
Syed Hussain
Rupak D Kulkarni
Jeffrey H Fair
Muhammad Mujtaba
Mike L Kueht

Publication Date

9-1-2022

Journal

Transplantation Direct

Abstract

Renal transplantation usually requires the administration of lifelong immunosuppressive therapy to minimize rejection.1 However, while these immunosuppressants are vital to graft protection, they increase the risk of infection and malignancy. Posttransplant lymphoproliferative disorder (PTLD), a troublesome combination of both, covers a wide range of lymphomas, most commonly B-cell type and has been described in pediatric and adult patients.2 Risk factors for PTLD include Epstein-Barr virus (EBV) infection and immunosuppression and the prevalence of adult kidney transplant PTLD is estimated to be 0.5% to 4%, with the highest incidences occurring within the first year of transplant.2-5 Although most cases are associated with EBV, EBV-negative PTLD has been described in the literature and is associated with worse outcomes.4

Hemophagocytic lymphohistiocytosis (HLH) is a sepsis-like inflammatory syndrome consisting of a hyperferritinemic immune response that can rapidly progress to multisystem organ failure (MOF).6 There are 2 types of HLH described: primary—associated with different genetic mutations—and secondary, which is more common and associated with infections and malignancies such as EBV and lymphomas.7 However, very few cases have been described associated with PTLD.6,8-12 Because HLH can present similarly to sepsis with MOF, it can be challenging for clinicians to discern and treat this condition, especially in transplant recipients.6 Here, we describe a challenging case of an adult kidney transplant recipient who developed EBV-negative PTLD with HLH.

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PMID: 35975023