Pediatric Hematology and Oncology - What Is Hemophilia

Hemophilia is a disorder that affects the natural blood clotting systems within our bodies. Clotting is the process in which platelets in our blood combine to transform blood from a liquid to a solid state.

Pediatric hematology and oncology specialists are forced to contend with not one but three types of hemophilia, with all types causing prolonged and often extensive bleeding, depending on the wound or injury. A child with hemophilia who experiences a cut will bleed for a longer period of time than a child with a normally functioning clotting mechanism. Those small cuts still clot eventually and aren't typically much of an issue. The larger concern for most patients, especially those with Type A and Type B hemophilia, is deep internal bleeding or bleeding within the joints due to trauma.

Pediatric Hematology and Oncology - What Causes HemophiliaYour body is pre-programmed with coagulation. This is the clotting mechanism that pools blood cells together to form a clot which stops the bleeding. Coagulation uses blood particles known as platelets along with plasma proteins to encourage clotting. Hemophilia is a defect or deficiency in one of these clotting factors. The type of deficiency determines the type of hemophilia a child has.

Type A - Type A Hemophilia is the most common type and is caused by a lack of clotting factor 8 (VIII)

Type B - This is the second most common type of deficiency and is caused by lack of clotting factor 9 (IX)

Type C - Type C is the least common, caused by a lack of clotting factor 11 (XI). It also has the fewest symptoms and is mild in comparison to the other types of hemophilia.

In most cases, hemophilia is inherited from the parents. The gene causing hemophilia A or B is located on the X chromosome, so it is only passed from mother to son. Most women with the defective gene are merely carriers and show no sign or symptom of the disorder. Type C can be passed to children by either parent, and can occur in both boys and girls.

Note that it is possible for both Type A and Type B to occur through a spontaneous gene mutation.

Pediatric Hematology and Oncology - How Is Hemophilia TreatedTreatments offered by specialists in pediatric hematology and oncology will differ based on the severity of the condition. Unfortunately, there is no cure, but with proper treatment, most children with the disease are able to lead normal, active and happy lives.

Drugs are sometimes used to stimulate the release of clotting factor. Likewise, some patients may receive clotting factors from donated blood or genetically engineered products. These are typically reserved for more severe cases of Type A and Type B. For Type C patients, pediatric hematology and oncology specialists rely on plasma in the U.S. to put a stop to bleeding episodes.