title: Hemophagocytic syndrome in children and adults.
abstract: Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is a heterogenic syndrome, which leads to an acute, life-threatening inflammatory reaction. HLH occurs both in children and adults, and can be triggered by various inherited as well as acquired factors. Depending on the etiology, HLH can be divided into genetic (i.e., primary) and acquired (i.e., secondary) forms. Among genetic HLH forms, one can distinguish between familial HLH and other genetically conditioned forms of HLH. Acquired HLH can be typically triggered by infections, autoimmune diseases, and malignancies. The most common symptoms of HLH are unremitting fever, splenomegaly, and peripheral blood cytopenia. Some severely ill patients present with central nervous system involvement. Laboratory tests reveal hyperferritinemia (often >10,000 μg/L), increased serum concentration of soluble receptor α for interleukin-2 (>2,400 U/L), hypertriglyceridemia, hypofibrinogenemia, coagulopathy, hyponatremia, hypoproteinemia, and elevated liver transaminases and bilirubin. Prognosis in HLH is very serious. Genetic HLH is always lethal if adequate therapy is not administered. Similarly, severe acquired cases often lead to death without appropriate treatment. Since HLH can be encountered by various specialists in the medical field, basic knowledge of this entity such as diagnostic criteria and treatment should be familiar to all physicians.
authors: Department of Pediatrics, Hematology and Oncology, Medical University of Warsaw, 00-576, Warsaw, Poland, iwona.malinowska@wum.edu.pl.
authors: Iwona
authors: I
authors: Malinowska
authors: Maciej
authors: M
authors: Machaczka
authors: Katarzyna
authors: K
authors: Popko
authors: Alicja
authors: A
authors: Siwicka
authors: Małgorzata
authors: M
authors: Salamonowicz
authors: Barbara
authors: B
authors: Nasiłowska-Adamska
mesh_terms: D000328:Adult; D000818:Animals; D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis* / Q000276:immunology; D002648:Child; D003602:Cytotoxicity, Immunologic / Q000235:genetics; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007239:Infections / Q000150:complications / Q000175:diagnosis / Q000276:immunology*; D053645:Interleukin-2 Receptor alpha Subunit / Q000097:blood; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000209:etiology / Q000276:immunology*; D008223:Lymphoma / Q000150:complications / Q000175:diagnosis / Q000276:immunology*; D054353:Perforin / Q000235:genetics; D022142:Secretory Vesicles / Q000235:genetics / Q000378:metabolism*; D014280:Triglycerides / Q000097:blood; D033921:Vesicular Transport Proteins / Q000235:genetics
chemical_list: C508017:IL2RA protein, human; D053645:Interleukin-2 Receptor alpha Subunit; C102161:LYST protein, human; C510468:PRF1 protein, human; D014280:Triglycerides; D033921:Vesicular Transport Proteins; D054353:Perforin