Acquired Hemophilia: A Life Threatening Bleeding Disorder
Acquired hemophilia is rare and life threatening disease which is caused due to excessive bleeding in the human body. Acquired hemophilia or AH is a rare autoimmune disorder which occurs due to excessive bleeding in the patients or persons who have personal and history of negative hemorrhages in the family. Auto immune disorders like Acquired Hemophilia or AH generally occurs in those human beings whose body’s immune system attacks the healthy tissues or cells of the human body by mistake. In the Acquired hemophilia disorder or AH, the human body produces inhibitors also known as antibodies that mostly attacks or affects the clotting factors of the human body. The body needs the clotting factors which are specialized proteins to clot the blood normally. The person or the individuals those who are suffering from this disease or disorder called as Acquired Hemophilia mainly develops complications like uncontrolled bleeding or abnormal bleeding in the soft tissues, muscles and skin.
The prevalence rate of acquired hemophilia is highly variable ranging from 0.045 per million in children to 14.7 per million adult population. It is an autoimmune disorder in which immunoglobulin G antibodies bind to the clotting factor VIII thereby drastically delaying the blood clotting time, which if not treated promptly may lead to fatal health complications. The mortality rate associate with acquired hemophilia is approximately 21%, caused due to incessant bleeding due to haemorrhage of internal organs.
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Currently, physicians worldwide are adopting a two pronged strategy for treating acquired hemophilia patients, eradicating the inhibitor against clotting factor VIII and control bleeding. Methyl prednisolone is started immediately alone or in combination with cytotoxic agents such as cyclophosphamide, azathioprine, cyclosporine and other immunosuppressant to reduce the inhibitor titer volume to facilitate blood clotting. At present, monoclonal antibodies such as rituximab and emicizumab are being investigated in phase 3 clinical trials for its therapeutic efficacy in reducing the inhibitor titre volume in the blood plasma, based on the positive results it is anticipated to become the first line therapy for treating acquired hemophilia.
The most important aspect is to control bleeding in patients hospitalized for haemorrhage of vital organs. Desmopressin is usually prescribed in minor bleeding cases with low inhibitor titre in blood plasma. FEIBA having half-life of 4-7 hours and NovoSeven RT with half-life of 2 hours are the bypassing agents, which are the first line therapy adopted in patients suffering with severe bleeding with high inhibitor titre volume. Administration of 200 U/kg of Obizur has shown positive clinical outcomes with therapeutic levels of factor VIII restored in the blood plasma showcasing 86% success rate. Obizur is anticipated to show promising results in the near future for its therapeutic efficacy in treating acquired hemophilia.
The key biopharmaceutical companies actively engaged in the manufacturing of drugs employed for the treatment of acquired hemophilia are Baxter Healthcare Corporation, BioXcel Corporation, Genetech, Inc., GlaxoSmithkline, Plc., Ferring B.V., Mylan N.V., Novo Nordisk, Sanofi SA, Shire, Plc. and Teva Pharmaceutical Industries Ltd.
Source: The insight partners