The emergency physician needs to be aware of major bleeding emergencies that can develop in patients with hemophilia. These patients require emergent factor replacement therapy and management by hemophilia specialists. Air transport to specialized centers should be considered for intracranial, intrathoracic, or intraabdominal bleeding, even if the patient appears "stable." Bleeding into the central nervous system (CNS) can occur spontaneously as well as with trauma. Any patient with hemophilia who complains of a new headache or any neurologic symptoms requires immediate factor replacement therapy followed by immediate computed tomographic (CT) scanning of the head. Spontaneous or traumatic bleeding into the neck, retropharynx, or pharynx has a high potential for airway compromise. Such bleeding can be spontaneous or precipitated by successful or unsuccessful placement of external jugular lines or other trauma. These patients require immediate factor replacement and immediate CT scanning to define the bleeding area. Airway management, including oral intubation, takes priority. If not preceded by factor replacement therapy, intubation must be followed immediately by factor replacement. Hemophilic patients with complaints of back, thigh, groin, or abdominal pain may have bleeding into the retroperitoneum. Bleeding into this potential space can be life-threatening because of the large potential area and the ability of the bleeding to dissect along fascial planes. Immediate factor replacement and CT scanning are indicated. Compartment syndromes result from muscle bleeds within the fascial compartments of the extremities. Complaints of pain and paresthesias and the findings of sensory, motor, or vascular deficits raise this possibility. After factor replacement therapy is initiated, the compartment pressure can be measured. Surgical fasciotomy may be required and needs to be done within 8 h after the onset of symptoms for best chance of full neurovascular recovery. The most common manifestation of hemophilia that will be encountered is the hemarthrosis. There may or may not be clinical evidence of an acute problem with the joint, but the patient can reliably report when bleeding is occurring. Prompt treatment of hemarthroses can prevent or reduce the long-term sequelae of hemophilic arthropathy. Patients with hemophilia should never receive intramuscular injections unless factor replacement is given and maintained for several days. Central lines, including femoral lines and external jugular lines, should not be placed in patients with hemophilia prior to factor replacement therapy; life-threatening bleeding can result. Arterial blood gases or lumbar puncture should not be performed on patients with hemophilia without coverage of factor replacement therapy. A compartment syndrome can result in the extremity, or epidural bleeding in the spinal canal.
The management of hemophilic bleeding depends on the type of hemophilia that is present, the severity of deficiency, the presence or absence of an inhibitor, and the location of bleeding. Each of these will be discussed separately.
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