Sites of Bleeding
Before reviewing treatment options, we must first start with identifying the sites at which bleeding might occur. Most children with hemophilia present as babies or toddlers, when crawling, walking, falling, and climbing occur. Presenting signs may include bleeding from circumcision, excessive bruising, mouth bleeding, bleeding from heel sticks, or swelling in a muscle or soft tissue. Bleeding can occur at various sites of the body with complications that depend on the site of bleeding. Bleeding sites fall into three categories: life-threatening, common, and other sites.
It is important to note that life-threatening bleeding must be treated as an emergency.
Older children with hemophilia have bleeding at sites similar to those of adults, with joint bleeding as the most common site. Young children, however, have bleeding related to their development stages and physical activities.
Central Nervous System (CNS)
Intracranial hemorrhage accounts for most deaths from bleeding among all age groups. Central nervous system bleeding can occur spontaneously or after any trauma.
Bleeding can occur around the spinal cord, usually after trauma. Neurologic sequelae can be severe.
Airway obstruction can occur from bleeding into and around the airway, including neck swelling and bleeding below the tongue. This usually occurs with trauma, but can result from medical or dental procedures.
Gastrointestinal Hemorrhage (GI)
Gastrointestinal bleeding can be seen in severe hemophilia and can occur in the absence of a demonstrable lesion. Patients with hepatitis, especially adults with varices, are at increased risk of GI bleeding.
Hemarthrosis (Joint Bleeding)
Bleeding into the joints is the most common site of bleeding in hemophilia. The primary site of bleeding is in the synovium that lines the joint and, if untreated, the bleeding progresses to fill the joint space. In toddlers, joint bleeding usually begins when the child has learned to walk or is learning to walk and is becoming more active.
The joints most frequently involved are ankles, knees, and elbows, but involvement of any joint can occur. Many patients experience repeated bleeding into the same joint or joints These are known as target joints and are defined as having had four or more bleeds during a 6-month period. Target joints have a risk developing chronic joint disease (hemophilic arthropathy).
Muscles are the second most frequent site of bleeding in hemophilia. With increased sports participation, sports-related injuries increase, including muscle hematomas from direct blows, which can cause large, deep muscle bleeds. Muscle bleeding can occur in any area of the body, (most frequently in extremities, with bleeding into some muscles that is more serious. For example, significant bleeding can occur in the thigh, resulting in major blood loss. Muscle bleeds in small spaces, such as the forearm or calf, can lead to compartment syndrome.
The iliopsoas is a large muscle, composed of the iliacus and psoas groups. The iliopsoas lies deep in the pelvis within the retroperitoneal cavity. With bleeding iliopsoas, large volumes of blood can be lost in the retroperitoneal space and lead to shock.
Nerve Compression (Compartment Syndrome)
Bleeding into a muscle compartment can compromise the neurovascular bundle. This should be considered with bleeding that occurs in the forearm, arm, calf and iliopsoas. Symptoms include pain with passive motion, pallor, decreased or absent pulses, and paresthesias.
Head trauma, even without symptoms, is considered an emergency that requires immediate treatment. Activities that carry the risk of falls and activities that may result in high collision or high-impact should be avoided because of bleeding risks.
Mouth bleeding is common among children with hemophilia and often results from falls or placing objects in their mouths. Adults with hemophilia can have mouth bleeding secondary to trauma, dental intervention, or biting their tongues. Frenulum tears are common sites of oral bleeding following trauma. Eruption and exfoliation of teeth are not usually problematic but can contribute to bleeding.
Blood in the urine can occur spontaneously among people with hemophilia. Most painless episodes have no known cause and do not involve significant blood loss. However, hematuria following trauma or associated with pain should be carefully evaluated, as should repeated episodes of hematuria.
Epistaxis (nose bleeding) is thought to be no more common for people with hemophilia than for others, but it can be difficult to manage for some patients.
Subcutaneous bleeding in hemophilia does not usually require treatment unless it occurs in an area that is confined or restricted, such as the orbit (around the eye), fingers, face, or neck.
Lacerations requiring sutures do require treatment among patients with hemophilia. Factor replacement might also be necessary at the time of suture removal.