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.