Hemophilia is a disorder of the blood clotting system. Because blood does not clot when it should, patients experience frequent and prolonged bleeding. The degree to which bleeding occurs depends in part on the severity of the disorder and trauma. Patients can have severe, moderate or mild hemophilia.

A hallmark of severe hemophilia is spontaneous bleeding into joint spaces and soft tissue. In these cases, bleeding occurs without any recognizable trauma. Spontaneous bleeds are most common in the knees, ankles, elbows and shoulders, but can occur anywhere. Repeated bleeding into the joints is called hemarthrosis. Spontaneous bleeding can occur in moderate hemophilia, but not as frequently.

Hemarthrosis usually begins after the child begins to walk. As the bleeding begins, the person may experience a warmth or tingling in the joint. As bleeding progresses, there is usually a feeling of stiffness, fullness, and pain. The joint swells and may be warm and tender. Bleeding into the joint limits the ability to move the joint. If not treated, hemarthrosis can cause chronic joint problems and arthritis.

In infants, severe hemophilia can also cause prolonged bleeding following circumcision. Bruising and muscle hemorrhages often occur in infants after injections. As the child becomes more active, excessive bruising occurs.

Symptoms of moderate hemophilia are the same as those for severe hemophilia with one exception. Spontaneous bleeding is rare in moderate hemophilia. When bleeding occurs, the person is usually able to identify some trauma that caused the event.

Patients with mild hemophilia may have prolonged bleeding with severe trauma or surgery. However, these patients may not experience any other problems or symptoms of the disorder. Many patients with mild hemophilia may forget they have the disorder until bleeding occurs due to a major event such as an accident or surgery.

The severity of the disease depends on the type of blood clotting factor that is missing. Symptoms are often more severe for Factor VIII deficiency (Hemophilia A) than for Factor IX deficiency (Hemophilia B).

The goal of treatment for hemophilia is to prevent and/or reduce the frequency of symptoms. Increasing factor levels to at least 5 percent of normal can cause symptoms of severe hemophilia to improve to the level of moderate or even mild hemophilia. This can be achieved with regular preventive infusions of factor.

First published February 1, 2000
Last updated December 2, 2002
Copyright © 2000 Accordant Health Services, Inc. All Rights Reserved.

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