Hemophagocytic Lymphohistiocytosisas the Initial Presentation of Epstein–Barr Virus‑Associated Post‑Transplant Lymphoproliferative Disorder in a Kidney Transplant Recipient
DOI:
https://doi.org/10.71749/pkj.169Keywords:
Epstein‑Barr Virus Infections, Kidney Transplantation/adverse effects, Lymphohistiocytosis, Hemophagocytic, Lymphoproliferative DisordersAbstract
Post-transplant lymphoproliferative disorder (PTLD) is one of the most serious malignancies following kidney transplantation and is strongly associated with Epstein-Barr virus (EBV) infection. Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome that only rarely presents as the initial manifestation of PTLD. We report the case of a 41-year-old kidney transplant recipient who presented with persistent fever and progressive systemic inflammation despite broad-spectrum antimicrobial therapy. The combination of bicytopenia, hyperferritinemia, hypertriglyceridemia, and splenomegaly, together with an HScore of 231 and an elevated soluble interleukin-2 receptor level, supported the diagnosis of HLH. A markedly elevated EBV viral load prompted investigation for PTLD. Although ¹⁸F-FDG PET/CT revealed no abnormal uptake, bone marrow biopsy established the diagnosis of EBV-positive monomorphic PTLD, diffuse large B-cell lymphoma type, with a germinal-centre B-cell phenotype and an isolated MYC rearrangement. Anakinra was initiated while the diagnostic investigation was ongoing, followed by attempted rituximab administration, obinutuzumab plus CHOP (Obino-CHOP) and reduction of immunosuppression. Clinical and laboratory improvement occurred in the context of these sequential interventions. The patient subsequently achieved complete haematological remission, documented by bone marrow assessment, with resolution of HLH and preservation of kidney allograft function.
This case highlights the importance of investigating the underlying cause of HLH in kidney transplant recipients. PTLD should be considered in patients with significant EBV viraemia, even in the absence of suggestive imaging findings. Management of hyperinflammation and investigation of its underlying cause should proceed in parallel to enable timely disease-specific treatment.
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The data supporting the findings of this case report are available from the corresponding author upon reasonable request.
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Copyright (c) 2026 Sandra Pereira, Maria Beatriz Bessa, Ana Beatriz Pereira, Josefina Santos, Maria La Salete Martins
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