Acute polymyositis associated with hemophagocytic lymphohistiocytosis syndrome

Background Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease in which an exaggerated but ineffective immune response leads to severe hyper inflammation. It commonly affects infants from birth to 18 months of age, but cases in older children and adults have also been reported. Key players in HLH are activated lymphocytes and histiocytes, they infiltrate all organs and secrete large amounts of cytokines. Cardinal symptoms are prolonged fever, hepatosplenomegaly, cytopenias and hemophagocytosis [1]. The aim of this case presentation is to focus on the unique presentations of HLH syndrome and to be aware of unusual presentations, so that we could diagnose early and treat it appropriately to ensure long term survival.