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Abstract: SA-PO0376

Two Cases of Hemophagocytic Lymphohistiocytosis-Associated AKI in Viral-Triggered Multiorgan Failure

Session Information

Category: Acute Kidney Injury

  • 102 AKI: Clinical, Outcomes, and Trials

Authors

  • Ohonba, Nosagie, Icahn School of Medicine at Mount Sinai, New York, New York, United States
  • Menon, Aiswarya, Icahn School of Medicine at Mount Sinai, New York, New York, United States
  • Sedlacek, Martin, Icahn School of Medicine at Mount Sinai, New York, New York, United States
Introduction

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening inflammatory syndrome caused by dysregulated cytotoxic T-cell and macrophage activation with excessive cytokine release. Renal injury is common and may result from ischemic acute tubular injury, cytokine-mediated tubular and endothelial damage, thrombotic microangiopathy, tumor lysis, sepsis, rhabdomyolysis, or multiorgan failure. We present two cases of viral-associated HLH complicated by severe AKI.

Case Description

Case 1: A 37-year-old woman with psoriasis and obesity presented with gastrointestinal symptoms and AKI. She quickly developed fever, shock, metabolic acidosis, and oliguria. Her course progressed to respiratory failure, liver injury, cardiomyopathy, cytopenias, hypertriglyceridemia, ferritin >30,000, and bulky lymphadenopathy. EBV PCR was positive, and lymph node biopsy showed EBER positivity without lymphoma, consistent with EBV-driven HLH. She was treated with anakinra, corticosteroids, and rituximab with improved inflammatory markers, but developed dialysis-dependent AKI.
Case 2: A 55-year-old woman with relapsed peripheral T-cell lymphoma and prior autologous stem cell transplant presented with recurrent fevers. She had parainfluenza and human metapneumovirus infection with hypotension, lactic acidosis, pancytopenia, ferritin >30,000, hypertriglyceridemia, splenomegaly, and AKI. She was diagnosed with HLH with progressive lymphoma burden, shock, DIC, transaminitis, and worsening AKI. Despite corticosteroids, anakinra, antimicrobials, transfusions, and lymphoma-directed therapy, she developed multiorgan failure requiring dialysis.

Discussion

HLH remains rare, with reported adult incidence rising from approximately 1 to 4 cases per million person-years. These cases highlight the renal spectrum of viral-associated HLH. HLH-associated AKI etiologies include cytokine storm, endothelial activation, capillary leak, tubular inflammation, and direct tubular injury. Shock, DIC, tumor lysis and multiorgan failure may further amplify renal injury. AKI occurs in approximately 30–60% of adult HLH cases and is associated with increased mortality, especially when renal replacement therapy is required. Early recognition of HLH in patients with fever, cytopenias, liver injury, hypertriglyceridemia, hyperferritinemia, and AKI is essential, as timely immunomodulation and trigger-directed therapy may improve renal and overall outcomes.