Acquired Haemophilia A: An Intriguing Disease

Mediterranean Journal of Hematology and Infectious Diseases
Maria Gabriella MazzucconiCristina Santoro

Abstract

. Acquired Haemophilia A is a rare acquired bleeding disorder caused by Factor VIII autoantibodies, which neutralise FVIII activity. These inhibitors differ from alloantibodies against FVIII, which can occur in congenital Haemophilia A after repeated exposures to plasma-derived or recombinant FVIII products. In most cases, the disease occurs suddenly in subjects without a personal or familiar history of bleedings, with symptoms that may be mild, moderate, or severe. However, only laboratory alterations are present in ~ 30% of patients. The incidence varies from 1 to 4 cases per million/year; more than 80% of patients are elderly, males and females are similarly affected. There is a small peak of incidence related to pregnancy in young women aged 20-40 years. The disease may be underdiagnosed in the elderly. The diagnostic algorithm is based on an isolated prolonged activated partial thromboplastin time, normal thrombin time, absence of Lupus Anticoagulant, and a mixing test that reveals the presence of an inhibitor: the finding of reduced FVIII activity and the detection of neutralising autoantibodies against FVIII lead to the diagnosis. The disease is idiopathic in 44%-63% of cases, while in the others etiological factors are ...Continue Reading

Citations

May 11, 2021·Frontiers in Medicine·Alina-Andreea ZimtaCiprian Tomuleasa
Aug 13, 2021·Haemophilia : the Official Journal of the World Federation of Hemophilia·Marika PorrazzoCristina Santoro

❮ Previous
Next ❯

Methods Mentioned

BETA
enzyme-linked immunosorbent assay

Related Concepts

Related Feeds

Blood Clotting Disorders

Thrombophilia includes conditions with increased tendency for excessive blood clotting. Blood clotting occurs when the body has insufficient amounts of specialized proteins that make blood clot and stop bleeding. Here is the latest research on blood clotting disorders.