Hypoprothrombinemia

Hypoprothrombinemia – General Information

Hypoprothrombinemia is a disorder which is characterized by a decreased level of prothrombin in the blood. Prothrombin is a protein which is absolutely vital during hemostasis and an insufficient quantity of this agent in the blood can result in a bleeding diathesis. Even though the muco-cutaneous bleeding which appears is not extremely severe and it can be associated with a large number of other problems in some cases, a prolonged hemorrhage can lead to the appearance of more serious problems as some more deep structures are also involved. This disease can be inherited or acquired and the second form also presents two different types: the first one appears as the result of an increased consumption of prothrombin and the second one is the result of a decreased production of prothrombin.  The acquired form of Hypoprothrombinemia is less severe and in most of the cases it is autoimmune; the reports have also shown that it is usually related to the lupus anticoagulant. Since now, studies have shown that the most common type of the acquired form of the disease is the vitamin K deficiency.  The other type of Hypoprothrombinemia, the inherited one, is quite rare and it presents two types. 
The good part about this disease is the fact that is quite rare and it affects only one person in 2 million. However, the risk of suffering from this disease is higher is the underdeveloped countries. Once the disease develops it can lead to the appearance of some severe complications and, in some cases, even death.

Hypoprothrombinemia – Symptoms

Even though this disease presents several forms and types which evolve quite differently, the initial phase is characterized by the same signs and symptoms in all the cases. The most common symptoms which have been reported in the case of Hypoprothrombinemia include: bleeding of the soft tissues, hemarthrosis and mucosal bleeding. More severe forms are characterized by the appearance of intracranial bleeding, hemorrhages, umbilical bleeding (in the case of young babies), muscle hematomas, menorrhagia and postoperative bleeding. If the disease is left untreated for a long period of time, the symptoms evolve and they will finally result in the development of anemia and all the problems associated with it. In the case of those patients who present this disease in association with the lupus anticoagulant, the first signs and symptoms which appear will indicate an autoimmune disease. In some rare cases, the patients who suffer form Hypoprothrombinemia can also present one or more of the following complications: epistaxis, prolonged bleeding in the case of an injury, a surgery or a tooth extraction, easy bruising, bleeding of the oral mucosa, hematochezia, melena, hematuria or hemathroses. There are also some side effects which can appear as the result of the excessive blood loss; the most common ones include anemia and pallor. There are some specific causes which can result in the appearance and the development of this disorder, especially its acquired type. The most common causes include thrombin deficiency and vitamin K deficiency; however, the disease can be induced by the administration of certain medicines.

Hypoprothrombinemia – Treatment

If you notice the appearance of any of the signs and symptoms described above, you should visit a doctor as soon as possible in order to obtain a sure diagnosis and to benefit from the best treatment. There are some specific tests which your doctor can recommend in order to detect the presence of Hypoprothrombinemia. In the first place, the doctor should carry through an assay in order to determine the presence of the factors which are responsible for the appearance of the second form of the disease, the acquired form. In addition to this test, he should also carry out some tests in order to determine a possible deficiency of vitamin K in the organism. These examinations can also provide important information in order to determine the presence of some complementary disorders or other complications. On the other hand, the tests which should be performed in the case of the inherited form of Hypoprothrombinemia are completely different, as this form of the disease affects young babies. In this case, the doctor has to undergo a head CT in order to measure the intracranial hemorrhage or a head ultrasonograph. In some conditions, the patient can also ask for a blood test, but this is not quite relevant.   

After the doctor establishes a sure diagnosis, he has to decide which form of treatment he should apply. However, the first measure which must be taken is the reduction and the controlling of the hemorrhage as soon as possible. After the patient’s condition is stabilized, the doctor will probably choose to administer him some prothrombin supplements, which are available in different forms and, in addition, there are a large number of products which contain prothrombin. Other forms of treatment which your doctor can choose from include the administration of intravenous immunoglobulin and the administration of steroids. In the case of a mild form of Hypoprothrombinemia, the doctor can choose to administer the patient just some vitamin K supplements. On the other hand, in the case of patients who experience severe forms of Hypoprothrombinemia, the doctor will probably choose a surgical intervention, in order to reduce hemorrhage.

The most important thing which a person who experiences this disease should also remember is the fact that he has to seek for medical care as soon as possible after the symptoms start to develop. After the treatment is completed, the doctor will probably make some recommendations in order to reduce the possibility of the reappearance of the disorder. In the first place, the patients should avoid further physical activities as much as possible; this recommendation should especially be taken into consideration in the case of those who had experienced head trauma and head hemorrhage. In order to obtain more information about their condition, those who had suffered from Hypoprothrombinemia should consult a hematologist, as this evolution of this disease can not always be predicted exactly and there are always some complications which can appear. However, the most important thing during the follow-up process is the fact that the patients have to be supported constantly by their family and friends.