title: Clinicohematologic Profile of Patients With Factor VIII Inhibitors: A Case Series.
abstract: Factor VIII (FVIII) inhibitors present major clinical challenge as a complication of hemophilia A in patients on treatment with FVIII concentrates and as acquired autoantibodies in patients without hemophilia A. We aimed to study the prevalence of FVIII inhibitors in Indian settings, risk factors involved in early development of inhibitors in patients with hemophilia, differences in their clinical behavior, and approach to treatment, in comparison to patients with acquired hemophilia. The overall prevalence of FVIII inhibitors in patients with severe hemophilia A was found to be 22.3%. Two cases of acquired hemophilia were reported. Due to heterogeneity of our study population, cases have been discussed individually. We observed that the early development of FVIII inhibitors in patients with hemophilia A is dependent upon an interplay of several risk factors that need to be studied in a multivariable analysis to bring out significant correlation with response to treatment. Also, they differ from patients without hemophilia A entirely in terms of presentation and management.
authors.affiliation: Department of Hematology, All India Institute of Medical Sciences, New Delhi, Delhi, India.
authors.forename: Neha
authors.initials: N
authors.lastname: Singh
authors.affiliation: Department of Hematology, All India Institute of Medical Sciences, New Delhi, Delhi, India.
authors.forename: Pravas
authors.initials: P
authors.lastname: Mishra
authors.affiliation: Department of Hematology, All India Institute of Medical Sciences, New Delhi, Delhi, India.
authors.forename: Seema
authors.initials: S
authors.lastname: Tyagi
authors.affiliation: Department of Hematology, All India Institute of Medical Sciences, New Delhi, Delhi, India.
authors.forename: H P
authors.initials: HP
authors.lastname: Pati
authors.affiliation: Department of Hematology, All India Institute of Medical Sciences, New Delhi, Delhi, India.
authors.forename: M
authors.initials: M
authors.lastname: Mahapatra
authors.affiliation: Department of Hematology, All India Institute of Medical Sciences, New Delhi, Delhi, India.
authors.forename: Tulika
authors.initials: T
authors.lastname: Seth
authors.affiliation: Department of Hematology, All India Institute of Medical Sciences, New Delhi, Delhi, India renusaxena@outlook.com.
authors.forename: Renu
authors.initials: R
authors.lastname: Saxena
mesh_terms: D000293:Adolescent; D000328:Adult; D001323:Autoantibodies / Q000097:blood*; D019774:Blood Coagulation Factor Inhibitors / Q000097:blood*; D002648:Child; D002675:Child, Preschool; D005169:Factor VIII / Q000037:antagonists & inhibitors*; D005260:Female; D006467:Hemophilia A / Q000097:blood* / Q000188:drug therapy; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D012307:Risk Factors
chemical_list: D001323:Autoantibodies; D019774:Blood Coagulation Factor Inhibitors; C078147:F8 protein, human; D005169:Factor VIII
keywords: FEIBA; acquired hemophilia; factor VIII inhibitors; hemophilia A; immune tolerance induction; recombinant factor VIIa