Renal Involvement in Hemophagocytic Syndrome

A rare cause of multi-organ failure

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare condition that complicates some rare inherited forms of immune deficiency, or occurring during the course of haematological (mainly non-Hodgkin lymphomas), infectious (herperviruses, bacteria or parasitosis) or autoimmune diseases (lupus, Still’s disease). It is defined by an acute over-activation of the immune system, involving particularly the macrophages and the cytotoxic T-cells. HLH induces a massive release of proinflammatory cytokines that generate fever, cytopenias, organomegaly, hepatopathy and coagulopathy. A multi-organ failure is usual in this situation, requiring admission in intensive care unit, with an overall mortality rate above 50% in many series. Acute renal failure is frequently observed among HLH patients, due to acute tubular necrosis, tubulo-interstitial inflammatory nephritis or specific glomerulopathy with nephrotic syndrome. Identification of this disease is a real challenge for the intensivist, especially in patients presenting like sepsis or septic shock, but positive diagnosis can modify therapeutic management by leading to initiation of chemotherapy or immunosuppressive drugs, according to the specific aetiology of HLH.