The mechanism that prevents the blood from flowing out of the vessel in bleeding in any part of the body is called coagulation. they are concentrated in the incision area. About 20 different clotting factors circulating in the blood begin to interact and convert the fibrinogen protein, which normally circulates freely in the blood, into fibrin. Fibrin combines with platelets and some other proteins to form a blood clot, and this clot closes the cut area in the vessel like a plug, preventing blood from flowing out of the vessel.
When there is a deficiency in one of the cells and substances that function in this system, bleeding and coagulation disorders occur and patients develop bleeding that does not stop even in tiny cuts. These diseases, which are defined as bleeding and coagulation disorders, are named and diagnosed according to platelet dysfunction or deficiency of factors that play a role in the coagulation mechanism. These diseases can be listed as follows. Acquired platelet dysfunction Congenital platelet dysfunction Disseminated intravascular coagulation disorder (DIC) Prothrombin deficiency Factor V deficiency Factor VII deficiency Factor X deficiency Factor XI deficiency Hemophilia Ahemophilia BIdiopathic thrombocytopenic purpura (ITP) Von Willebrand's disease Glanzmann's disease may develop due to disorders.
What are the symptoms of bleeding and coagulation disorders?
Common symptoms are generally observed in all of these diseases. All symptoms are that the bleeding that stops for a short time in normal individuals does not stop for a long time or that traumas that do not cause significant bleeding or bruising in normal individuals cause severe bleeding or bruising on the skin in these patients.
The bruising on the skin that develops after traumas develops as a result of bleeding from the capillaries under the skin. In large amounts of bleeding, swelling develops under the skin along with bruising. Common symptoms can be listed as follows.
Excessive bleeding or easy bruising after injuries Severe bleeding from a minor cutBleeding gumsTooth � Bleeding that doesn't stop after transplantation Nosebleeds that stop for no reason and very difficult Bleeding in the wound stops and starts again Swelling and pain in the joints due to intra-articular hemorrhages. Bleeding that does not stop after circumcision in boys. Heavy menstrual bleeding that can last for a very long time in girls
What is hemophilia?
Hemophilia is a bleeding and coagulation disorder that has 3 types, A, B, and C, and is caused by the deficiency of coagulation factors. This disease is an X-linked recessive disease. That is, women are carriers and only occur in men. It usually occurs in childhood.
The diagnosis is made by the suspicion of the physician and by performing diagnostic tests in families who apply to the doctor with nosebleeds, non-stop bleeding, especially swelling and pain in the knee joints in boys. Girls are usually carriers. The disease is not seen in sons born to a non-hemophilic mother and a father with hemophilia. For diagnosis, coagulation tests and tests to measure the levels of factors in the blood are performed.
The treatment of hemophilia is made according to the severity of the disease. Patients with 5-30% of the normal coagulation factor level are classified as mild hemophilia, 1-5% patients with moderate hemophilia, and patients with less than 1% of the normal factor level are classified as severe hemophilia.
The missing factor in the treatment is replaced. For this, factor concentrates obtained from human blood with high technology are used. Patients with mild hemophilia are usually not treated. Precautionary factor concentrates may be given before a major operation. In severe and moderate hemophilia, a protective factor concentrate is administered to patients at regular intervals. A therapeutic dose of factor concentrates is given before operations such as surgery or tooth extraction. With the home treatment method, some patients can use the medicine themselves.
After long-term factor therapy, antibodies developed against exogenous factors, called inhibitors, appear in the blood of patients. When these antibodies are formed, the missing factor is broken down by the inhibitor and cannot be used by the body as soon as it is given to the body from the outside. This is one of the biggest problems in the treatment of hemophilia patients.
Idiopathic tr ombocytopenic purpura (ITP)
Thrombocytopenic purpura (ITP) of unknown cause is an autoimmune disease caused by a disorder of the immune system. Our immune system detects foreign substances (bacteria, viruses, cancer cells, etc.) and marks them with proteins called antibodies, and these cells are destroyed by white blood cells. However, in autoimmune diseases, for unknown reasons, the body's own cells are perceived as foreign and destroyed by white blood cells. In
ITP, the immune system perceives the platelets as foreign and the platelets are destroyed in the spleen and other lymphoid organs. Normally, the platelet count is determined as 150,000-450,000/mm3 in the blood count. However, in this disease, the platelets in the blood drop below 100,000 and usually small, reddish purplish petechial (pin-tip) hemorrhages appear on the skin. When the platelet count is below 30,000/mm3, patients must be treated, otherwise fatal bleeding may occur. In some patients, platelet count returns to normal with a single course of treatment and may not require further treatment, but repeat treatments may be required in some patients.
In the treatment, cortisone treatment is primarily applied. Cortisone therapy suppresses the activated immune system and prevents platelet destruction. Due to the side effects of cortisone treatment, immunoglobulin treatment can also be applied for the same purpose. It should not be ignored that these treatments will suppress not only the immune system, but also the whole system, and that the patients will be prone to infections and diseases during the treatment. Splenectomy (removal of the spleen), that is, surgical removal of the spleen, is of great benefit to patients in severe cases where no results can be obtained despite this drug treatment.
Platelet Function Disorders
In these diseases, while platelet counts are normal, platelet functions are impaired. While these diseases can occur congenitally at an early age, they can sometimes be acquired in adulthood. Acquired platelet dysfunction may occur in response to certain drugs, diseases, and certain foods. Platelet count and coagulation factors in patients Although the surface is normal, dysfunction should be suspected when bleeding disorders are observed. In treatment, cutting off the drug or food that causes the disease usually leads to a gradual recovery of functions. Platelet replacement should be performed in cases of acute bleeding.
Diffuse intravascular coagulation disorder (DIC)DIC is a very noisy condition and difficult to treat. It is a bleeding-coagulation disorder that develops against some diseases and conditions rather than a disease. These can be listed as follows. Sepsis (an infection in the bloodstream)Surgery and traumaCancer Serious complications of pregnancy and childbirthSnake poisoning Excessive blood clotting begins in the capillaries. As a result of this uncontrollable blood coagulation, the clotting factors in the circulation are exhausted, the platelets become unable to function, and this time, bleeding disorder develops and excessive bleeding occurs. The only treatment for this condition, also called consumption coagulopathy, is replacement therapy with blood and blood products.
Today, bleeding and coagulation disorders are in the group of diseases that are easily treated. If you believe that you or your child have the symptoms mentioned, do not neglect to make an appointment with Hematology specialists working within Florence Nigtingale Hospitals Group.
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