Catastrophic hemophagocytic lymphohistiocytosis in a young man with nephrotic syndrome

Abstract

Background: In its early stage, necrotizing fasciitis mimics a milder cutaneous infection, such as cellulitis, and is an uncommon but potentially fatal complication of nephrotic syndrome. It may trigger an uncontrolled and catastrophic immune response, such as hemophagocytic lymphohistiocytosis (HLH).

Case report: A 19-y-old man presented with steroid-resistant nephrotic syndrome and rapidly progressing Escherichia coli monomicrobial necrotizing fasciitis with bacteremia. The conditions developed one day after steroid therapy, leading to multiple organ dysfunction syndrome. A provisional diagnosis of HLH was promptly made, based upon the patient's fever, unremitting shock, marked pancytopenia, hyperferritinemia, hypofibrinogenemia, and the typical histiocytic hemophagocytosis in pleural effusion. Despite aggressive medical treatment and organ support, the patient died 8 days after transfer to our intensive care unit. Final bone marrow examination confirmed the diagnosis of HLH.

Conclusions: Although nephrotic syndrome associated with E. coli infection is common, this is the first reported case of E. coli monomicrobial necrotizing fasciitis with bacteremia resulting in HLH in a patient with nephrotic syndrome.

Keywords: Hemophagocytic lymphohistiocytosis; Necrotizing fasciitis; Nephrotic syndrome.