Difference between revisions of "Acquired hemophilia"
(Created page with "'''Other Names:''' Acquired haemophilia Acquired hemophilia is a bleeding disorder that interferes with the body's blood clotting process. Although the condition can affect...")
(Tag: 2017 source edit)
Latest revision as of 15:41, 1 August 2020
Other Names: Acquired haemophilia
Acquired hemophilia is a bleeding disorder that interferes with the body's blood clotting process. Although the condition can affect people of all ages, it generally occurs in older people (the median age of diagnosis is between 60 and 67 years). Acquired hemophilia occurs when the body's immune system attacks and disables a certain protein that helps the blood clot . About half of the cases are associated with other conditions, such as pregnancy, autoimmune disease, cancer, skin diseases, or allergic reactions to medications.
Signs and symptoms
Signs and symptoms include prolonged bleeding, frequent nosebleeds, bruising throughout the body, solid swellings of congealed blood (hematomas), hematuria, and gastrointestinal or urologic bleeding. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.
80%-99% of people have these symptoms
- Anemia(Low number of red blood cells or hemoglobin)
- Autoimmune antibody positivity
- Prolonged partial thromboplastin time
- Reduced factor VIII activity
- Subcutaneous hemorrhage(Bleeding below the skin)
30%-79% of people have these symptoms
- Intramuscular hematoma
5%-29% of people have these symptoms
- Gastrointestinal hemorrhage(Gastrointestinal bleeding)
- Hematuria(Blood in urine)
- Post-partum hemorrhage(Bleeding post-delivery)
1%-4% of people have these symptoms
- Intracranial hemorrhage(Bleeding within the skull)
- Joint hemorrhage(Bleeding within a joint)
Any acute or recent onset of bleeding symptoms in a patient with no previous history of bleeding, especially in elderly or post-partum patients, and an unexplained isolated prolonged activated partial thromboplastin time (aPTT) suggest the diagnosis of AHA, and need further investigation. The first step to distinguish between factor deficiency and the presence of an inhibitory substance is to perform a mixing test, in which patient plasma and normal plasma are mixed and aPTT measured; correction of prolonged aPTT suggests a factor deficiency while persistent prolongation indicates the presence of an inhibitor. Prolongation of the aPTT in a mixture of patient and normal plasma after a 1-2 h incubation compared to an immediate mix is typical of FVIII autoantibodies, as FVIII inhibitors are time and temperature-dependent.
The diagnosis is confirmed by the subsequent identification of reduced FVIII levels with evidence of FVIII neutralising activity (titrated using the Bethesda assay or the Nijmegen modification)
Treatment is aimed at controlling bleeding episodes and addressing the underlying cause of the condition. Patients with acquired coagulation factor inhibitors should be treated jointly with haemophilia centres experienced in the management of inhibitors. Initial treatment consists of four steps: Prevention of bleeding events, avoiding or minimizing actions that might provoke bleeding such as intramuscular injections, invasive procedures and use of antiplatelet and nonsteroidal anti-inflammatory agents. Treatment of the underlying disorder. Therapy of bleeding Antibody eradication
The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.
- Antihemophilic factor (recombinant), Fc fusion protein (Brand name: Eloctate) antihemophilic factor (recombinant), Fc fusion protein (Eloctate) was approved for the treatment of adults and children with Hemophilia A (congenital Factor VIII deficiency) for control and prevention of bleeding episodes, perioperative management, and routine prophylaxis to prevent or reduce the frequency of bleeding episodes.
NIH genetic and rare disease info
Acquired hemophilia is a rare disease.