Macrophage activation syndrome (MAS) is a serious complication of childhood systemic inflammatory disorders that is thought to be caused by excessive activation and proliferation of T lymphocytes and macrophages. Recent findings in hemophagocytic lymphohistiocytosis, a disease that is clinically similar to MAS, highlight the possible pathogenetic role of a defective function of perforin, a prot...

Macrophage Activation Syndrome is a rare and potentially fatal disease, to be considered in patients with a SIRS like clinical presentation. Falling ESR and hyperferritinemia in the appropriate clinical setting can be the greatest clues. We report a case in which a female admitted with fever and rash, initially had systemic inflammatory response syndrome (SIRS) like clinical presentation, but e...

There are no laid down criteria for the diagnosis of MAS. The diagnostic criteria laid down by Histiocyte Society to diagnose HLH are generally applied to the diagnosis of MAS.5 These consist of clinical features (fever, hepato-splenomegaly) along with laboratory features of cytopenia involving at least 2 cell lineages in the peripheral blood (Hb < 9 gm/dl, neutrophils < 1,000/mm3, and platelet...

IMacrophages are important constituents of mononuclear phagocyte system, earlier known as reticulo-endothelial system. Tissue macrophages are innate immune cells with well-established roles in the primary response to pathogens, particularly abundant in capillary walls of lung (alveolar macrophages), Liver (Kupffer cells), spleen, brain, connective tissues, serous cavities (peritoneal macrophage...

BACKGROUND Kawasaki disease is an acute febrile vasculitis of childhood. Macrophage activation syndrome is a rare life threatening complication. CASE CHARACTERISTICS 4-year-old boy with Kawasaki Disease treated with intravenous immunoglobulins. OBSERVATION He developed encephalopathy, hepatosplenomegaly and pancytopenia. Blood investigations and bone marrow aspiration suggested macrophage a...

Macrophage Activation Syndrome (MAS) occurs as a severe life-threatening complication of several chronic rheumatic diseases. It is more frequent with systemic onset juvenile arthritis and adult onset Still's disease.1 It can be primary, infection related, malignancy associated or autoimmune3. We report a case of Macrophage Activation Syndrome presenting as pyrexia of unknown origin (PUO) and pa...

PURPOSE OF THE REVIEW One of the most perplexing features of systemic-onset juvenile rheumatoid arthritis is the association with macrophage activation syndrome, a life-threatening complication caused by excessive activation and proliferation of T cells and macrophages. The main purpose of the review is to summarize current understanding of the relation between macrophage activation syndrome an...

Introduction. Adult-onset Still?s disease is a rare inflammatory disorder of unknown etiology. It can be complicated by macrophage activation syndrome, potentially life-threatening condition. While syndrome and adult-onset share similar features, early recognition very difficult in clinical praxis. Case outline. We report young woman, whose illness was presented suddenly, with spiking fever, so...