Hemophagocytic syndrome in systemic lupus erythematosus

Abstract

Introduction. A hemophagocytic syndrome is a life-threatening disorder, which develops as a result of the activation of macrophages. It may be divided into two distinct types. A primary hemophagocytic syndrome is caused by genetic mutations while secondary may occur in the course of infections, malignancies, or autoimmune disorders. When constituting a complication of autoimmune diseases, the hemophagocytic syndrome is commonly referred to as macrophage activation syndrome. Objective. We report a case of secondary hemophagocytic syndrome in the course of systemic lupus erythematosus to draw attention to this condition, which may often be under-diagnosed in patients with autoimmune disorders. Case report. A 27-year-old woman was admitted to the Department of Dermatology with symptoms resembling an exacerbation of systemic lupus erythematosus. The hemophagocytic syndrome was diagnosed by clinical manifestation, i.e., fever, hemophagocytosis in bone marrow aspirate, neutropenia, increased ferritin level, and hypertriglyceridemia. Conclusions. Hemophagocytic syndrome should be taken into consideration in systemic lupus erythematosus patients as it can mimic an acute flare of the disease.