A close insight to factor VIII inhibitor in the congenital hemophilia A.
INTRODUCTION
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.


AREAS COVERED
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.
['Tabriznia-Tabrizi|Shamsoreza|S|', 'Gholampour|Marzie|M|', 'Mansouritorghabeh|Hassan|H|']
D000818:Animals D001402:B-Lymphocytes D002450:Cell Communication D005169:Factor VIII D020022:Genetic Predisposition to Disease D006467:Hemophilia A D006801:Humans D007518:Isoantibodies D011379:Prognosis D011994:Recombinant Proteins D012307:Risk Factors D012720:Severity of Illness Index D016176:T-Lymphocyte Subsets
factor VIII inhibitor Hemophilia A inhibitor development environmental risk factor genetic risk factors