Abstract
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening condition. It is an immune-mediated disease that has a wide range of causes, elicits a hyperinflammatory response, and results in multiple organ damage. Clinical presentations vary, and in some cases, jaundice occurs as the first symptom. CASE SUMMARY: We report the case of a 71-year-old female patient who presented with jaundice. She was admitted to our hospital because of the occurrence of "jaundice for half a month", and upon examination, obstructive jaundice with choledocholithiasis and gallstones was suggested. Cholecystectomy and choledocholithotomy were performed. However, the jaundice did not improve after surgery. We found splenomegaly, cytopenia, hypertriglyceridemia, hypofibrinogenemia, and elevated ferritin. Bone marrow biopsy revealed hemophagocytosis. Later, cardiac arrest occurred when she returned 3 wk after the surgery. We considered that HLH was triggered by septic shock. The patient's condition deteriorated rapidly, with multiple organ dysfunction and severe gastrointestinal bleeding. Corticosteroid therapy and symptomatic treatment failed to save her life. CONCLUSION: Jaundice rarely presents as the first symptom in HLH patients. The HLH in this case was triggered by septic shock with jaundice as the first symptom. Clinicians should try hard to reduce missed diagnoses and misdiagnoses.