Hemophagocytic Lymphohistiocytosis Following Peritoneal Dialysis-Associated Peritonitis with Neurological Involvement: A Case Report

Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal hyperinflammatory condition resulting from dysregulated immune activation. While HLH may be primary or secondary to triggers such as infection, its development following peritoneal dialysis (PD)-associated peritonitis is extremely rare. We reported a case with neurological involvement, underscoring diagnostic and therapeutic challenges in dialysis patients. Case Presentation: A 32-year-old woman with end stage renal disease on automated PD for six years presented with fever and rigors while receiving treatment for Staphylococcus aureus PD peritonitis. Despite negative PD cultures and clinical improvement, she developed progressive delirium and hemodynamic instability. Neuroimaging and cerebrospinal fluid analysis were normal. Laboratory results revealed bicytopenia (Hb 7.2 g/dL, platelets 109 × 10⁹/L), hyperferritinemia (33,500 µg/L), elevated LDH (2,644 U/L), and hypertriglyceridemia (2.14 mmol/L). Bone marrow biopsy confirmed hemophagocytosis, fulfilled five of eight HLH-2004 diagnostic criteria. Treatment with intravenous immunoglobulin (IVIG) and dexamethasone led to clinical recovery. Her course was complicated by fungal catheter-related bloodstream infection and atrial fibrillation, prompting a temporary switch to sustained low-efficiency daily dialysis (SLEDD) before resumption of PD. Discussion: Diagnosis HLH in dialysis patients is challenging due to overlap features with sepsis or uremic encephalopathy. Mortality rate reported up to 40% without timely treatment (1). Neurological symptoms occurs in 30-73% of cases (2). To date, only three cases of HLH triggered by PD peritonitis have been reported and none with central nervous system (CNS) symptoms (3,4,5). Our case is the first to report neurological involvement. While HLH treatment often includes corticosteroids, IVIG, and additional immunosuppressants (2,6), our patient responded well to corticosteroids and IVIG alone, suggesting that less intensive immunotherapy may be effective in dialysis-related HLH. Conclusion: HLH should be considered in dialysis patients with unexplained systemic and neurological symptoms. Early diagnosis and tailored immunotherapy, even without cytotoxic agents, can yield favorable outcomes.