Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening hyperinflammatory syndrome characterized by uncontrolled immune activation. It is most often associated with infections, malignancies, or autoimmune disorders such as adult-onset Still's disease (AOSD). Percutaneous nephrolithotomy (PCNL) is regarded as a safe and routine procedure for the treatment of large renal calculi. To date, HLH has not been described as a postoperative complication of PCNL.
A 47-year-old man underwent elective PCNL for a 10 × 22-mm stone in the left renal pelvis. The operation was uneventful. Four hours postoperatively, he developed severe oral and rectal bleeding, thrombocytopenia, and rapidly increasing liver enzymes. Computed tomography revealed no surgical complications. Due to progressive clinical deterioration with suspected thrombotic microangiopathy, the patient was transferred to the intensive care unit. Bone marrow biopsy demonstrated hemophagocytosis. Treatment with corticosteroids, interleukin-1 receptor antagonists, intravenous immunoglobulins, and plasma exchange resulted in rapid clinical and laboratory improvement. Further evaluation revealed previously undiagnosed AOSD as the underlying trigger.
HLH represents a diagnostic challenge, as it can mimic common postoperative complications such as sepsis or bleeding disorders. Surgical trauma may act as a trigger in predisposed patients with occult autoinflammatory disease. Early interdisciplinary collaboration is essential to initiate immunomodulatory therapy and prevent fatal outcomes.
This case illustrates HLH as a rare but severe complication following PCNL. Unexplained cytopenia, liver dysfunction, and bleeding after urological surgery should prompt consideration of HLH, even in patients without a known history of autoimmune disease.
International journal of surgery case reports. 2026 Jun 02*** epublish ***
Johann Gerd Bierlein, Martin Kurosch, Rene Mager, Daniel Sasca, Axel Haferkamp, Maximilian Pjk Brandt
Department of Urology and Pediatric Urology, Johannes-Gutenberg University Medical Center, Mainz, Germany., Department of Hematology, Oncology and Pneumology, University Medical Center, Mainz, Germany.