Hemophagocytic lymphohistiocytosis histiocytosis research.docVIP

 PAGE \* MERGEFORMAT 17 Hemophagocytic lymphohistiocytosis histiocytosis research Keywords:: histiocytosis non - Langerhans - cell Hemophagocytic lymphohistiocytosis histiocytosis (HLH) belongs to the first Histiocytosis Ⅱ , macrophage-related diseases accounted for the majority. This paper reviews the recent research and clinical trials of HLH diagnosis and treatment strategy, and an overview of its biology, immunology and pathophysiological characteristics. hLH, including two difficult to distinguish the subtypes. (1) primary HLH: namely, familial hemophagocytic lymphohistiocytosis histiocytosis (FHL, FHLH or FEL). (2) secondary HLH: that the infection-associated hemophagocytic syndrome (IAHS or VAHS) and malignant associated hemophagocytic syndrome (MAHS) [1. The incidence and epidemiology 1 Children with primary HLH (FHL) of the annual incidence rate is about 0.12/10 10000. There have been reports from around the world. FHL is autosomal recessive inheritance, most of the age of onset of a very small, with a median age of onset was 2.9 months [2]. A report related to the incidence of at least two members of the nine homes, there are four families were incidence of amp;quot;6 months. Parents, next of kin marriages accounted for 24%, a positive family history accounted for 49%. Secondary HLH exact incidence is unknown. From 1979 to 1995, the literature 219 cases have been reported from newborn to 18 years old LAHS, half are in order from Japan, China and Taiwan [3]. Half of the age amp;quot;3 years old. 13% of the existence and immune deficiencies or immunosuppressive therapy-related primary disease. IAHS trigger factor was up to see the EB virus, EB virus-associated HLH there exists an obvious immunological imbalances and poor prognosis [4]; other viruses including human herpes virus type 6 [5], cytomegalovirus, adenovirus, parvovirus, varicella 1-zoster virus, herpes simplex virus, Q fever virus and measles virus and cells, fungi, protozoa, and so on.