An Easily Overlooked Disease: Hemophagocytic Syndrome

Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is a heterogenic syndrome, which leads to an acute, life-threatening inflammatory reaction. We report a case of rapid death due to HLH induced by chronic, active Epstein-Barr virus (EBV) infection.

Appropriate laboratory tests, abdominal ultrasonography, and cervical lymph node biopsy.

Hemoglobin and platelet counts decreased, fasting triglyceride increased to 2.32 mmol/L, ferritin > 1,500 ng/mL, soluble CD25 (interleukin-2 receptor) > 2,400 U/mL, and abdominal ultrasound indicated splenomegaly, meeting the diagnostic criteria of HLH. A biopsy of the left cervical lymph node revealed chronic, active EBV infection.

HLH is likely under-recognized, and mortality remains high, especially in adults; thus, prompt diagnosis and treatment are essential.