Hemophagocytic Syndrome in Children and Adults

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• 作者：Iwona Malinowska ; Maciej Machaczka…
• 关键词：Hemophagocytic lymphohistiocytosis ; Hemophagocytic syndrome ; Children ; Adults
• 刊名：Archivum Immunologiae et Therapiae Experimentalis
• 出版年：2014
• 出版时间：October 2014
• 年：2014
• 卷：62
• 期：5
• 页码：385-394
• 全文大小：774 KB
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• 作者单位：Iwona Malinowska (1)
  Maciej Machaczka (2) (3)
  Katarzyna Popko (4)
  Alicja Siwicka (1)
  Ma?gorzata Salamonowicz (1)
  Barbara Nasi?owska-Adamska (5)
  
  1. Department of Pediatrics, Hematology and Oncology, Medical University of Warsaw, 00-576, Warsaw, Poland
  2. Division of Hematology, Department of Medicine at Huddinge, Karolinska Institutet, M54, SE-141 86 Stockholm, Sweden
  3. Medical Faculty, University of Rzeszow, Rzeszow, Poland
  4. Department of Laboratory Medicine and Pediatric Immunology, Medical University of Warsaw, Warsaw, Poland
  5. Institute of Hematology and Transfusiology, Warsaw, Poland
  
• ISSN：1661-4917

文摘

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.