Acquired hemophilia in pediatrics: A systematic review

Abstract

Acquired hemophilia A is a rare but potentially life-threatening hemorrhagic disorder caused by the development of autoantibodies directed mostly against coagulation factor VIII. Acquired hemophilia is frequently associated with several underlying conditions such as malignancy, autoimmune disorders, drug reactions, and pregnancy, although the pathogenesis remains undetermined (idiopathic) in up to 50% of reported cases. The disorder occurs most commonly in the elderly and only rarely affects pediatric patients, who might however experience severe, and sometimes life-threatening, hemorrhage. The maternal transplacental transfer of the autoantibody to the neonate occurs very rarely and also in this circumstance it may be associated with clinically significant bleeding. The management of acute bleeding and the inhibitor eradication are the mainstay of the treatment. The outcome in pediatric patients seems more favorable than in adults because the inhibitors usually resolve more quickly and in a higher rate of patients. The epidemiology, diagnosis, clinical course, and management of this hemorrhagic disorder in children will be addressed in this systematic review.

A child with acquired haemophilia

“…The mortality rate is 8-22% with most deaths occurring in the first few weeks after presentation [4,5]. These autoantibodies are usually of polyclonal IgG 1 and IgG 4 subclasses, leading to neutralization and/or accelerated clearance of FVIII from plasma in nonhemophilic persons [1,2,5]. The age distribution of AHA is biphasic with a small peak between 20-30 years due to postpartum FVIII antibodies and the major peak in patients aged 68-80 years [3].…”

“…2, June 2014 Case report patients [3]. The mortality rate is 8-22% with most deaths occurring in the first few weeks after presentation [4,5]. These autoantibodies are usually of polyclonal IgG 1 and IgG 4 subclasses, leading to neutralization and/or accelerated clearance of FVIII from plasma in nonhemophilic persons [1,2,5].…”

Exsanguinating bleeding following tooth extraction in a 12-year-old girl

“…The overall incidence of AHA is estimated to be 0.2-4 per million/year. However, the incidence in children is only 0.045 per million/year [1,2]. In adults, there is a major peak in the elderly; there is also a small peak in the 20 to 30-year age bracket that is due to pregnancy-related AHA.…”

“…A recent review found 28 cases of paediatric AHA in the literature. Of these, 23 had skin bleeding and haematomas, only one had haemarthrosis and three had life-threatening intracranial haemorrhage [1]. A surgery-triggered diagnosis of AHA was not described in any of these cases.…”

Pitfalls in hemostasis exploration, a case report of a girl with Henoch-Schönlein type vasculitis

“…(1,2) Lupus anticoagulants (LA) belong to the heterogenous group of antibodies directed against negatively charged phospholipids, with a prevalence of approximately 5% in adults and more than 20% in children and adolescents. (3) LA is a double misnomer: it is only seldom associated to systemic lupus erythematosus and almost never causes bleeding disorders.…”