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Ethan S Sen, Colin G Steward, Athimalaipet V Ramanan
Haemophagocytic syndrome, or haemophagocytic lymphohistiocytosis (HLH), is a hyperinflammatory disorder characterised by uncontrolled activation of the immune system. It can result from mutations in multiple genes involved in cytotoxicity or occur secondary to a range of infections, malignancies or autoimmune rheumatic diseases. In the latter case, it is also known as macrophage activation syndrome (MAS). Characteristic features are persistent fever, hepatosplenomegaly, petechial/purpuric rash, progressive cytopenias, coagulopathy, transaminitis, raised C reactive protein, falling erythrocyte sedimentation rate, hypertriglyceridaemia, hypofibrinogenaemia and extreme hyperferritinaemia often associated with multi-organ impairment...
March 2017: Archives of Disease in Childhood
Takahiro Yasumi, Masayuki Hori, Eitaro Hiejima, Hirofumi Shibata, Kazushi Izawa, Hirotsugu Oda, Kouhei Yoshioka, Kenji Nakagawa, Tomoki Kawai, Ryuta Nishikomori, Osamu Ohara, Toshio Heike
Haemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of immune dysregulation and is classified as primary or secondary according to the underlying aetiology. The treatment strategies recommended for these two groups differ substantially; however, it is thought to be impossible to predict the underlying causes of HLH using conventional laboratory tests. Recent studies show that serum levels of soluble interleukin-2 receptor (sIL2R) and ferritin are useful for differentiating some forms of HLH...
August 2015: British Journal of Haematology
Scott W Canna, Edward M Behrens
Cytokine storm syndromes (CSS) are a group of disorders representing a variety of inflammatory causes. The clinical presentations of all CSS can be strikingly similar, creating diagnostic uncertainty. However, clinicians should avoid the temptation to treat all CSS equally, because their inciting inflammatory insults vary widely. Failure to identify and address this underlying trigger results in delayed, inoptimal, or potentially harmful consequences. This review places the hemophagocytic syndromes hemophagocytic lymphohistiocytosis and macrophage activation syndrome within a conceptual model of CSS and provides a logical framework for diagnosis and treatment of CSS of suspected rheumatic origin...
April 2012: Pediatric Clinics of North America
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