Acquired haemophilia is a severe bleeding disorder that occurs not only in men but also in women. Symptoms of this acquired bleeding disorder often appear suddenly, can be turbulent, and can be life-threatening. What are the causes and symptoms of acquired haemophilia? What is the treatment?

Acquired haemophilia(acquired haemophilia- AH) is a severebleeding disorderthat occurs in both men and women. Hemophilia is most often diagnosed in elderly people, aged 60-80 years. The disease is detected in 1 - 1.5 people per 1 million general population.

Acquired haemophilia - causes

Acquired haemophilia is caused by autoantibodies - known as a circulating anticoagulant or a FP inhibitor. VIII. - which impair the function of coagulation factor VIII. In acquired haemophilia, in contrast to congenital haemophilia, this factor is properly produced by the body.

It is not known why these antibodies appear, but certain diseases may contribute to this - most often cancer or autoimmune conditions such as rheumatoid arthritis and systemic lupus. Patients who often receive blood products are also at risk.

Acquired haemophilia is also a rare complication of childbirth and the puerperium.

Acquired haemophilia - symptoms

  • extravasation of blood under the skin, usually extensive, spontaneous and traumatic
  • massive bleeding from the mucous membranes (including urinary and genital tract bleeding)
  • massive bleeding from wounds after surgery and tooth extraction
  • extensive and painful intramuscular bleeding which may lead to damage

The first symptom of acquired haemophilia is often a massive haemorrhage

In contrast to classical haemophilia, spontaneous bleeding into the joints is very rarely observed in the course of acquired haemophilia. Soft tissue bleeding predominates in AHA. Intracranial bleeding is the most dangerous because it is usually not possible to stop it.

Acquired haemophilia - diagnosis

Blood tests are performed when acquired haemophilia is suspected. Factor VIII is reduced and autoantibodies are found to impair coagulation factor VIII function. In addition, there is an extended partial timeactivated thromboplastin (APTT). On the other hand, the prothrombin time and bleeding time are normal.

Acquired haemophilia - treatment

The goal of treating patients with acquired haemophilia is to inhibit bleeding and eliminate autoantibodies.

In acquired haemophilia, as opposed to congenital haemophilia, coagulation factor concentrates containing factor VIII are not administered as this increases the production of antibodies and may worsen the patient's condition and bleeding. To stop bleeding, recombinant factor VIII or a concentrate of activated prothrombin complex factors are used.

In order to remove autoantibodies in the bloodstream, immunosuppressants are used - most often prednisone and cyclophosphamide.

Acquired haemophilia - prognosis

Mortality associated directly or indirectly with bleeding complications reaches 10-22 percent.

from wounds after surgical operations and tooth extraction procedures as well as extensive and painful intramuscular hematomas

Category:

Cardiology