Clinical profile, etiology, and outcome of hemophagocytic lymphohistiocytosis associated with histiocytic necrotizing lymphadenitis

Hemophagocytic lymphohistiocytosis(HLH)is a life-threatening clinical syndrome characterized by aberrantly activated macrophages and cytotoxic T cells,leading to mul-tiple organ failure[1].The main manifestations included recurrent fever,cytopenia,liver dysfunction,and sepsis-like syndrome.Physicians should be fully aware of HLH since early diagnosis and management may prevent irreversible organ failure and subsequent death.Treatment protocols,such as HLH-94 based on etoposide(VP 16),are commonly used in clinical practice,especially in familial HLH[2]and malignancy-associated HLH[3].