HISTORY. Question the patient, parents, or caregiver about any history of prolonged bleeding episodes, either spontaneous or following any injury in the patient or family. Determine the patient's age at diagnosis and the specific nature of the bleeding problem; in the case of a child, determine the family member's relationship to the patient.

PHYSICAL EXAMINATION. Note hematomas from subcutaneous and intramuscular bleeding. Tissue over the bleeding site is hard, raised, and dark purple. The hemorrhage extends from this center concentrically, with each successive outer circle becoming lighter in color. Intramuscular bleeding usually spreads within a single fascial space. Fever or pain may occur, with or without the skin discoloration.

Monitor the patient for frank or occult hematuria, melena, or hematemesis. Bleeding disproportionate to the extent of a traumatic injury is characteristic of hemophilia. Typically, the bleeding is an intermittent oozing type that develops over several hours or days after the injury or procedure. Wound healing is often delayed.

When the patient's extremities are moved, note joint pain and swelling caused by hemarthro-sis, or spontaneous or trauma-induced bleeding into the joint or synovial cavity. Acute hemarthrosis is often preceded by a warm tingling sensation in the affected joint. If absorption of the blood from around the periarticular structures is incomplete, the remaining blood can cause chronic inflammation of the synovial membranes. Other long-term clinical sequelae of hemarthrosis include impaired joint mobility, bone deformity and demineralization, and stunted growth. Approximately 40% of hemophilia patients have splenomegaly; there have been some reported cases of spontaneous splenic rupture.

PSYCHOSOCIAL. Hemophilic patients and their families contend with the challenges of a chronic illness and the constant threat of life-threatening hemorrhage. Children often feel isolated

Diagnostic Highlights

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