Home Topics Coagulation DisordersHypoprothrombinemia

Inherited and Acquired Hypoprothrombinemia

Hypoprothrombinemia is a blood disorder that results from a deficiency of prothrombin. Also known as Factor II, prothrombin is blood plasma protein essential for blood clotting. A lack of prothrombin results in blood clotting problems, meaning the body will have trouble stopping bleeding after an injury.

In serious cases of hypoprothrombinemia, patients also suffer from internal hemorrhaging, particularly in the gastrointestinal system.

Hypoprothrombinemia Causes

Here are some of the parameters that put people at a higher risk of developing hypoprothrombinemia:

Hypoprothrombinemia is more prevalent in insulated populations (i.e. people that don’t have much, if any, contact with those of other cultures or nations).
Hypoprothrombinemia can arise as the result of some other underlying medical problem.
Some people inherit this disorder at birth. However, this is a rare occurrence, occurring in approximately one in 2 million people.
This condition may develop as a side effect to some types of medications.

Vitamin K deficiency at birth can cause a person to develop the disease, though this is rarely a problem in the United States where vitamin K injections are typically given to newborns.

Hypoprothrombinemia Diagnosis

Though inherited hypoprothrombinemia is an extremely rare disorder, doctors can typically diagnose this condition shortly after birth, as a newborn’s inability to recover from birth trauma (i.e. umbilical cord detachment) tends to result in excessive bleeding.

Diagnosing forms of hypoprothrombinemia that patients develop later in life usually occurs after a patient has noticed some type of bleeding problem, such as nosebleeds or menstrual periods that will not resolve in a normal period of time. Any abnormal bleeding or bruising should raise warning flags and put individuals and their care providers on alert for underlying problems, such as hypoprothrombinemia.

Women who suffer from lupus may also develop this coagulation disorder due to the effect of some antibodies on the body’s systems. This can result in a serious secondary disorder known as lupus anticoagulant-hypoprothrombinemia syndrome. If you are a woman who suffers from lupus, ask your doctor about these risks.

Symptoms of Hypoprothrombinemia

The process the body undergoes to clot blood involves a complex series of steps, or coagulation factors. These factors are typically denoted using Roman numerals I through XII (1 through 12). Each of these factors represents a specific, vital protein necessary to the clotting process.

A deficiency or interruption in any of these processes can result in a serious clotting problem. Specifically, when the bloodstream has insufficient levels of prothrombin, a patient develops hypoprothrombinemia, a condition characterized by the following symptoms:

an increased susceptibility to bruising
bleeding gums
excessive or prolonged bleeding with injuries or surgery.

Hypoprothrombinemia Treatment

The treatments for hypoprothrombinemia will vary, depending on the precise cause of the condition. One method of treating this disorder revolves around administering occasional plasma (with purified prothrombin complexes) infusions to the patient. These infusions restore the body’s supply of prothrombin to promote blood clotting.

If a vitamin K deficiency is the cause of hypoprothrombinemia, then vitamin K supplements will be prescribed, as well.

Hypoprothrombinemia suffers must take great care to avoid injuries, especially head injuries, to prevent the possibility of excessive bleeding. Therefore, another key part of the treatment for this disease is to avoid risky activities and, if possible, surgery. If surgery must be performed on a person with hypoprothrombinemia, the patient must also receive copious infusions of prothrombin.

Hypoprothrombinemia Prognosis

The risks of complications and even death associated with hypoprothrombinemia depend on the level of prothrombin deficiency in a specific case. However, most people with bleeding disorders can lead relatively normal lives. Sufferers simply need to be vigilant about their physical condition and handle any potential problems quickly. Any incident that may cause internal bleeding must be dealt with quickly to ensure that no ongoing threat goes unresolved.

Resources

Lee Culvert, Teresa Norris RN, Thomson Gale. Gale, Detroit (2007). Hypoprothrombinemia. Gale Encyclopedia of Children’s Health. Retrieved September 24, 2007, from the Health Line Web site: http://www.healthline.com/galecontent/coagulation-disorders/3#hypoprothrombinemia.

Harper, James L., M.D. (2006). Hypoprothrombinemia. Retrieved September 24, 2007, from the Health Line Web site: http://www.healthline.com/galecontent/coagulation-disorders/3#hypoprothrombinemia.