Extranodal NK/T cell lymphoma-associated hemophagocytic syndrome: where do we stand?

Hemophagocytic lymphohistiocytosis (HLH), first reported as “familial hemophagocytic reticulosis” by Farquhar JW in 1952, is a rare life-threatening disorder characterized by overwhelming immune and inflammation activation, and “cytokine storm” (1-5). HLH can be roughly categorized as primary HLH (pHLH), which is inherited, or secondary HLH (sHLH) occurring in a context of infection, autoimmune diseases or malignancies.