Haemophilia A - Signs and Symptoms

Signs and Symptoms

Characteristic symptoms vary with severity. In general symptoms are internal or external bleeding episodes, which are called "bleeds". Patients with more severe hemophilia suffer more severe and more frequent bleeds, while patients with mild hemophilia typically suffer more minor symptoms except after surgery or serious trauma. Moderate hemophiliacs have variable symptoms which manifest along a spectrum between severe and mild forms.

If forceps or vacuum extraction are used in vaginal births, the first signs of hemophilia may be severe head bruising or hematomas or even intracranial hemorrhage. Prolonged bleeding from a circumcision wound or a venepuncture or heelprick is another common early sign of hemophilia. These signs may lead to blood tests which indicates hemophilia. In other patients, especially those with moderate or mild hemophilia a later trauma will lead to the first serious bleed.

Hemophilia leads to a severely increased risk of prolonged bleeding from common injuries, or in severe cases bleeds may be spontaneous and without obvious cause. Bleeding may occur anywhere in the body. Superficial bleeding such as those cause by abrasions, or shallow lacerations may be prolonged and the scab may easily be broken up due to the lack of fibrin, which may cause re-bleeding. While superficial bleeding may be troublesome, by far the most serious sites of bleeding are:

• joints
• muscles
• digestive tract
• brain

The muscle and joint hemorrhages are indicative of hemophilia, while digestive tract and cerebral hemorrhages are also germane to other coagulation disorders.

Though typically not life threatening, joint bleeds are one of the most serious symptoms of hemophilia. Repeated bleeds into a joint capsule can cause permanent joint damage and disfigurement resulting in chronic arthritis and disability. Joint damage is not a result of blood in the capsule but rather the healing process. When blood in the joint is broken down by enzymes in the body, the bone in that area is also degraded. Therefore, this exerts massive amounts of pain upon the person afflicted with the disease.