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High-Yield Facts

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  • Aggressive treatment of hemophilia patients with head trauma is imperative as signs and symptoms of intracranial bleeding may be delayed. Even though initial imaging studies may be normal, factor replacement is indicated and careful monitoring of the patient is crucial to detect subtle changes in mental status.

  • Patients with hemophilia and inhibitors remain challenging management cases and are best cared for in conjunction with a hematologist.

  • Treatment of bleeding episodes in von Willebrand patients may include desmopressin (DDAVP), cryoprecipitate, or factor VIII concentrates rich in von Willebrand factor (vWf).

  • Treatment options for ITP are based on the clinical severity of bleeding. Consultation with a hematologist is indicated for patients with bleeding complications or platelet counts <20,000.

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Hemophilia

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Hemophilia is an X-linked recessive disorder of coagulation caused by deficiency of factor VIII (hemophilia A) or factor IX (hemophilia B), occurring in one in 5000 men.1 The percentage of factor present determines the severity of disease. Six to forty percent factor activity levels denotes mild disease with no tendency for spontaneous hemorrhage and bleeding occurs usually only with surgery or severe trauma. Two to five percent implies moderate disease with bleeding following mild trauma. Less than 1% is severe disease with proclivity to spontaneous hemorrhage. Two-thirds of male patients with hemophilia have severe disease. In both hemophilia A and B, the prothrombin time (PT) is normal and partial thromboplastin time (PTT) is prolonged. The same types of bleeding occur in both factor VIII and factor IX deficiency. Bruising, hemarthroses, and intramuscular hematomas predominate. Intracranial hemorrhage is less common but can be devastating. It is important to listen to the patient and their parents as the initial presentation of bleeding may not be dramatic.

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Acute Hemarthrosis
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Knees, elbows, ankles, hips, and shoulders are the most commonly affected joints (Fig. 105-1). Older patients may be aware of a bleed prior to the onset of pain and swelling, whereas younger patients may present with new onset limp or limited range of motion. It is generally agreed that even if joint bleeding cannot be confirmed, treatment is indicated. This philosophy is based on the potentially crippling sequelae of hemarthrosis. Intraarticular bleeding provokes a strong synovial inflammatory reaction causing erosion of the cartilage, synovial hypertrophy, and friability. Muscle atrophy around the joint leads to instability, which increases the likelihood of more frequent hemarthroses. Unless treated early and adequately, repeated bleeding into a “target joint” can lead to complete cartilaginous destruction causing secondary osteoarthritis. Joint swelling that is persistent and associated with fever may indicate a septic joint. Aspiration preceded by appropriate factor replacement may be necessary but should only occur after discussion with the child's hematologist. Joint aspiration is not recommended for most cases of bleeding.

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FIGURE 105-1

A 5-year-old with severe factor VIII deficiency and acute left knee hemarthrosis.

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