Case report “a dangerous black box:” idiopathic hemophagocytic lymphohistiocytosis in adult patients—a case report and review of the literature

HIGHLIGHTS

SUMMARY

Clinically, HLH patients present with fever, splenomegaly, and a sepsis-like syndrome. Although sepsis can trigger HLH, it is important to identify underlying associated HLH as soon as possible, because immunosuppressive therapy is required to suppress the cytokine storm of HLH, a strategy that is not present systematically in sepsis guidelines. HLH is a condition better known in children than in adults, although awareness regarding HLH in the adult population is increasing. Te incidence of HLH in the pediatric population is 1 in 300.000, whereas the real incidence of HLH in the adult . . .

 



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