1 INTRODUCTION
Hemophagocytic lymphohistiocytosis (HLH) is a severe and potentially lethal disorder characterized by overwhelming immune activation and inflammation 1. Primary HLH mostly occurs in childhood and is caused by genetic defects leading to impaired function of natural killer cells and cytotoxic T cells which lead to bouts of increased cytokine release and severe inflammation. Secondary HLH is encountered in association with some viral infections, auto-inflammatory/autoimmune diseases, or lymphoma 2. For both primary and secondary HLH, there exist excessive activation of T cells and macrophages and therefore lead to “cytokine storm” as well as multiple organ disorder. So the most important thing is to control the inflammatory cytokine storm in the treatment of HLH. The conventional therapy included steroid, intravenous immunoglobulin (IVIG) and immunosuppressant. Recently, several blood purification methods have been reported to be successfully practiced as adjuvant therapy to resolve hypercytokinamia complicated with HLH 3, 4.
In the current study, we firstly reported two cases of secondary HLH children received hemoadsorption therapy with HA330-II perfusion column.