Hereditary bleeding disorder

Characterized by greatly prolonged coagulation time

Results from deficiency of specific clotting factors

Hemophilia A (classic hemophilia: affects more than 80% of hemophiliacs, resulting from factor VIII deficiency

Hemophilia B (Christmas disease): affects 15% of hemophiliacs, resulting from factor IX deficiency

Most common X-linked genetic disease

Occurs in about 1.25 of 10,000 live male births

Incurable

A low level or absence of the blood protein necessary for clotting causes disruption of normal intrinsic coagulation cascade.

This produces abnormal bleeding, which may be mild, moderate, or severe, depending on the degree of protein factor deficiency.

After a platelet plug forms at a bleeding site, the lack of clotting factors impairs formation of a stable fibrin clot.

Hemorrhage isn’t always immediate; delayed bleeding is common.

Acquired immunologic process

Hemophilia A and B inherited as X-linked recessive traits

Spontaneous mutation

Familial history of bleeding disorders

Prolonged bleeding with circumcision

Concomitant illness

Pain and swelling in a weight-bearing joint, such as the hip, knee, or ankle

With mild hemophilia or after minor trauma, lack of spontaneous bleeding, but prolonged bleeding with major trauma or surgery

Moderate hemophilia producing only occasional spontaneous bleeding episodes

Severe hemophilia causing spontaneous bleeding

Prolonged bleeding after surgery or trauma or joint pain in spontaneous bleeding into muscles or joints

Signs of internal bleeding, such as abdominal, chest, or flank pain; episodes of hematuria or hematemesis; and tarry stools

Activity or movement limitations and need for assistive devices, such as splints, canes, or crutches

Hematomas on extremities, torso, or both

Joint swelling in episodes of bleeding into joints

Limited and painful joint range of motion (ROM) in episodes of bleeding into joints