title: A close insight to factor VIII inhibitor in the congenital hemophilia A.
abstract: BACKGROUND
  Hemophilia A (HA) has an X-linked pattern of inheritance and is the most common of the hemorrhagic disorders. HA is caused by a decreased or deficiency of the functional clotting factor VIII (FVIII) and effects 1 in 5000-10,000 male births. The common treatment for hemophilia is replacement therapy by plasma-derived or recombinant FVIII. Approximately 20-30% of people with a severe type of HA develop an inhibitor and this phenomenon is the main challenge in the management of these patients. Genetic factors and environmental determinants contribute to inhibitor development. Here, the roles of various genetic and environmental factors such as the type of FVIII concentrate used, the number of exposure days, and peak treatment time will be discussed in detail. It seems this information is helpful for hematologists.
  
  
  METHODS
  A literature review was done in January 2016 on PubMed and Scopus using the following keywords:' h(a)emophilia A & factor VIII inhibitor', 'h(a)emophilia A & factor VIII alloantibody', 'h(a)emophilia A & inhibitor'. There was no time limitation; however, there was an English language limitation placed on the articles selected. Expert commentary: Influential genetic and environmental factors in developing inhibitors have been discussed. Most of the risk factors are related to previously untreated patients with hemophili.
authors.affiliation: a Haematology , Mashhad University of Medical sciences (MUMS) , Mashhad , Iran.
authors.forename: Shamsoreza
authors.initials: S
authors.lastname: Tabriznia-Tabrizi
authors.affiliation: a Haematology , Mashhad University of Medical sciences (MUMS) , Mashhad , Iran.
authors.forename: Marzie
authors.initials: M
authors.lastname: Gholampour
authors.affiliation: b Allergy Research Center, Ghaem hospital, School of Medicine , Mashhad University of Medical Sciences , Mashhad , Iran.
authors.forename: Hassan
authors.initials: H
authors.lastname: Mansouritorghabeh
mesh_terms: D000818:Animals; D001402:B-Lymphocytes / Q000276:immunology / Q000378:metabolism; D002450:Cell Communication; D005169:Factor VIII / Q000235:genetics / Q000276:immunology* / Q000627:therapeutic use; D020022:Genetic Predisposition to Disease; D006467:Hemophilia A / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics / Q000276:immunology*; D006801:Humans; D007518:Isoantibodies / Q000276:immunology*; D011379:Prognosis; D011994:Recombinant Proteins / Q000009:adverse effects* / Q000276:immunology* / Q000627:therapeutic use; D012307:Risk Factors; D012720:Severity of Illness Index; D016176:T-Lymphocyte Subsets / Q000276:immunology / Q000378:metabolism
chemical_list: D007518:Isoantibodies; D011994:Recombinant Proteins; D005169:Factor VIII
keywords: Hemophilia A; environmental risk factor; factor VIII inhibitor; genetic risk factors; inhibitor development