LAUSR

BACKGROUND Haemophagocytic syndrome (HPS) is rarely seen in patients with acute pancreatitis (AP). HPS as a complication of AP in patients without any previous history has not been elucidated. CASE SUMMARY A 46-year-old man was admitted for symptom of persistent abdominal pain, nausea, and vomiting for 2 d after heavy drinking. During hospital stay, he suddenly developed skin rash and a secondary fever. The laboratory findings revealed progressive pancytopenia, abnormal hepatic tests, and elevation of serum triglyceride, ferritin, and lactate dehydrogenase levels. However, apparent bacterial or viral infections were not detected. He was also possibly related to autoimmune diseases because of positive expression of various autoimmune antibodies and no remarkable past history. Finally, the bone marrow examination showed a histiocytic reactive growth and prominent hemophagocytosis, which resulted in a diagnosis of HPS. Unexpectedly, the patient responded well to the immunosuppressive therapy. CONCLUSION HPS is a very rare extrapancreatic manifestation of AP. The diagnosis relies on bone marrow examination and immunosuppressive therapy is effective. For AP with skin changes, the possibility of HPS should be considered during clinical work.