How is acquired hemophilia treated?

In one study, 35 patients with acquired hemophilia and severe bleeding were treated with a combination of cyclophosphamide, prednisolone, large volume immunoadsorption, IVIG, and FVIII, and treatment appeared to achieve rapid remission in the vast majority of patients.

How is factor IX deficiency treated?

Standard treatment is infusion of factor IX concentrates to replace the defective clotting factor. The amount infused depends upon the severity of bleeding, the site of the bleeding, and the size of the patient.

How is factor deficiency treated?

How rare factor deficiencies are treated depends on the missing protein. Some can be treated with factor concentrates that replace the missing clotting factor. Others are treated with fresh frozen plasma, which is the liquid part of whole blood, or antifibrinolytics, which slow the breakdown of blood clots.

How is clotting factor deficiency treated?

Specific treatment options for acute bleeds in individuals with factor VII deficiency include recombinant factor VII, prothrombin complex concentrates, and fresh frozen plasma. Antifibrinolytics such as aminocaproic acid can help alleviate the bleeding symptoms.

What is acquired VIII deficiency?

Acquired factor VIII (FVIII) deficiency, or acquired hemophilia A (AHA), is a rare autoimmune disorder involving antibody-mediated depletion of coagulation FVIII, leading to severe, life-threatening bleeding.

What causes factor IX deficiency?

Hemophilia B, also called factor IX (FIX) deficiency or Christmas disease, is a genetic disorder caused by missing or defective factor IX, a clotting protein. Although it is passed down from parents to children, about 1/3 of cases are caused by a spontaneous mutation, a change in a gene.

What activates factor IX?

Factor IX is activated as the result of the cleavage of two peptide bonds, Arg145 and Arg180–Ile181, to form a two-chain enzyme. The chains are linked by a disulfide bond, and thus, the Gla domain remains associated with the protease domain and maintains phospholipid association capability.

What is the average lifespan of a person with hemophilia?

In severe hemophilia, all-cause mortality did not change significantly during 1977 to 1999. During this period, it exceeded mortality in the general population by a factor of 2.69 (95% confidence interval [CI]: 2.37-3.05), and median life expectancy in severe hemophilia was 63 years.

Are there any cures for factor IX deficiency?

Highly purified factor IX (FIX) concentrates are now available. These include monoclonal antibody–purified plasma-derived FIX (pdFIX; Immunine and Mononine) and recombinant FIX (rFIX).

Are there any inhibitors for factor XI deficiency?

Inhibitors in patients with hemophilia and factor XI deficiency are discussed separately. (See “Inhibitors in hemophilia: Mechanisms, prevalence, diagnosis, and eradication” and “Factor XI (eleven) deficiency”, section on ‘Inhibitor development’ .)

How is factor IX deficiency treated in hemophilia B?

After a single intravenous infusion of the vector, circulating FIX levels increased to 1% to 6% of normal in all 10 patients over a median of 3.2 years. Four of the seven patients previously on factor replacement were able to discontinue it, and the others were able to decrease the dose.

What to do if you have factor X deficiency?

Consultation in patients with factor X deficiency include hematologists and, in cases of congenital factor X deficiency, genetic counselors. No dietary restrictions are necessary in individuals with factor X deficiency. Patients are advised to limit alcohol consumption to reduce the risk of liver disease.