What are Bleeding Disorders?

The term ‘bleeding disorder’ refers to several different medical conditions that cause the blood to clot poorly or cause continuous bleeding. Bleeding disorders may also be called coagulopathy, abnormal bleeding or clotting disorders. People who have bleeding disorders will bleed longer than normal, healthy persons and the disorders can result from defective blood vessels or from abnormalities in the blood. Such abnormalities may be in the substances that make the blood clot or in the blood platelets.

When the body is injured or harmed in any way the blood forms into clumps or ‘clots’ and in this way controls the bleeding i.e. the blood is changed from a liquid to a solid. This clotting process is controlled by twenty different clotting factors in the blood which form a substance called fibrin which stops bleeding. If certain factors in the blood are missing the blood can’t clot normally and bleeding occurs for a longer time. This kind of bleeding can be very mild or severe depending on the disorder.

Bleeding disorders have the following symptoms:

• Excessive bleeding

• Excessive bruising

• Easy bleeding

• Nose bleeds

• Abnormal menstrual bleeding

What Causes Bleeding Disorders?

Bleeding disorders may be present at birth and are inherited for example hemophilia or they may be a feature of certain illnesses vitamin K deficiency or severe liver disease. Bleeding disorders may also be caused by or treatments such as anticoagulant drugs or prolonged use of antibiotics). They are caused by a number of different diseases such as:

• von Willebrand's disease - an inherited blood disorder affecting between 1% and 2% of the population.

• Immune system diseases like allergic reactions to medications, or reactions to an infection.

• A blood cancer like leukemia.

• Liver disease

• Bone marrow problems (including those caused by chemotherapy and radiation).

• Disseminated intravascular coagulation - a condition associated with child bearing, cancer, or infection, where the clotting system doesn’t function properly.

• Pregnancy-associated eclampsia.

• Organ transplant rejection to Hemophilia A and B, which are inherited blood disorders.

• Exposure to snake venom

• Antibodies (immune system proteins) that destroy blood clotting factors

• Medicines, such as aspirin, heparin, warfarin, and drugs used to get rid of blood clots.

What are the Risks of Bleeding Disorders?

• Scarring of the joints or joint disease.

• Vision loss from bleeding into the eye.

• Chronic anemia (low number of red blood cells in the body) from blood loss.

• Neurologic or psychiatric problems

• Death, which may occur with large amounts of blood loss or bleeding in critical areas, such as the brain.

Types of Bleeding Disorders

The blood may show several kinds of problems with its blood clotting mechanisms such as:

• Idiopathic Thrombocytopenic Purpura – in which the blood has too few platelets.

• Dysfunctional platelets

• Von Willebrand’s Disease (hereditary disease where clotting factors are missing)

• Hemophilia (hereditary disease where clotting factors are missing)

Idiopathic Thrombocytopenic Purpura (ITP)

Idiopathic thrombocytopenic purpura is a kind of bleeding disorder for which the cause is usually not known but it may be caused by certain illnesses or drugs such as those used in chemotherapy. In fact, the word ‘idiopathic’ means ‘unknown’. In this disorder the blood doesn’t have enough platelets as these are destroyed by antibodies created in the person’s blood. It is sometimes called ‘immune thrombocytopenic purpura’.

If a person has ITP, all their blood cells are normal except for their blood platelets. These are the cells that act to close minor cuts and wounds and form blood clots. When there are too few platelets in the blood the body is easily bruised and if an injury occurs bleeding continues for a long time.

Sometimes tiny red spots called petechiae occur on the skin or the person may have nosebleeds that are difficult to stop, or bleeding in the intestines.

Who gets ITP?

There are two types of ITP one affecting adults and the other children. ITP doesn’t run in families.

How is ITP Treated?

The aim of treatment is to increase the number of platelets in the blood but this doesn’t cure the disease. Prednisone is usually prescribed for several weeks or more than a month in some cases but when the medication is stopped the blood platelets may start to decrease.

Sometimes the spleen is removed since it is the organ that produces the antibodies that kill the blood platelets. If the patient is a fairly healthy individual this is not as serious as it sounds.

Platelet Dysfunction

There are several factors that may lead to a dysfunction of the blood platelets. A dysfunction means the platelets don’t work normally or work below the level that leads to good health.

  Uremia may cause the platelets to become dysfunctional as a consequence of either acute or chronic kidney failure. Uremia occurs when excess wastes in the blood are not excreted in the urine and cause the blood to become toxic.

These ‘poisons’ interfere with the function of blood platelets and cause nausea, vomiting, weight loss, metallic taste in the mouth and odor on the breath, itching, a frosty look to the skin, muscle pain and twitching, increased blood pressure, swelling and water retention, and mental confusion. A doctor will know if you have uremia if an exam reveals kidney failure.

The aim of treatment is to correct kidney function or improve the function of the platelets. Dialysis or successful kidney transplant may be used. If you are anemic you may be given a red blood cell transfusion and erythropoietin hormone replacement since anemia also affects the platelet count of the blood.)

  Drugs like desmopressin (DDAVP), cryoprecipitate (concentrated blood clotting proteins), and conjugated estrogens such as Premarin can reduce bleeding time.

  Hepatocellular disease or liver disease like hepatocellular carcinomas and adenomas (cancers) can cause the blood platelets to become dysfunctional and lead to a bleeding disorder. The levels of platelets in the blood are decreased because the spleen becomes enlarged and traps them causing them to be unable to re-enter the bloodstream.

  Dysproteinemia causes increased blood levels of abnormal proteins which interfere with the normal functioning of the blood platelets. Diseases like multiple myeloma and macroglobulinemia cause abnormal proteins to be released into the blood. The aim of treatment is to address the underlying cause but plasmapheresis, or ‘plasma exchange’, may be used to control bleeding.

  Myeloproliferative disorders or diseases of the bone marrow can cause bleeding disorders due to platelet dysfunction since platelets are produced in the bone marrow.

  Drugs like as aspirin and other nonsteroidal anti-inflammatory agents (Advil, Excedrin) can cause platelet function to become inhibited since the stop the platelets from clotting at the site of a wound. Other drugs like heparin or sulfa antibiotics such as Bactrim also cause decreased platelet function.

Von Willebrand’s Disease

Von Willebrand’s Disease is a bleeding disorder that is genetic in origin in other words we inherited it from our parents. In von Willebrand’s disease the blood either has low levels of a specific protein that causes the blood to clot or the protein is dysfunctional or both.

The protein implicated in von Willebrand’s disease is known as the von Willbrand factor and if it is missing or doesn’t work properly it will cause bleeding to last longer when the body is injured. This is because this protein acts like a glue to hold the blood platelets together at the site of a wound or injury. The von Willebrand’s factor also contains a protein called clotting factor VIII that helps with clotting of the blood.

Types of von Willebrand Disease

Three major types of von Willebrand disease exist and they are:

• Type 1: This is where there are low levels of the von Willebrand factor and possibly low levels of factor VIII. It is a mild form of the disease and also the most common form. Type 1 occurs in about three of four people with the disease.

• Type 2: In type 2 the von Willebrand factor is defective and doesn’t work properly. Type 2 is divided into 2A and 2B and each is treated differently.

• Type 3: In type 3 there is no von Willebrand factor at all and very low levels of factor VIII. Type 3 is severe but very rare.

Hemophilia

Two types of hemophilia occur: Type A and Type B. Both of these are caused by either low levels or the complete absence of a blood protein needed for proper blood clotting. Hemophiliacs with type A lack the blood clotting factor VIII and people with type B lack factor IX. In the United States there are about 20 000 people living with hemophilia. About 85% have hemophilia A.

How severe the clotting disorder will be depends on the deficiency of clotting factors but about 70% have severe hemophilia.

The most common complication in patients with hemophilia is chronic joint disease caused by bleeding into the joint and life-threatening hemorrhage is an ever present risk. In the United States treatment for hemophilia is ‘on demand’ so they are given a factor replacement once bleeding symptoms are experienced and these bleeds can result in severely impaired joints.

Some European countries treat hemophiliacs by periodic infusion (prophylaxis) of factor replacement regardless of their bleeding status. This approach maintains the factor level high enough that bleeding, joint destruction, and life-threatening hemorrhage can be avoided.

Whatever type of bleeding disorder you have it is important for you to understand what is happening in your body and to take the necessary steps to protect your health by avoiding the risks of prolonged bleeding and infection. If you show any of the above symptoms tell your doctor immediately. If you have a bleeding disorder talk to your doctor about the best way to manage your bleeding disorder and ensure your continued well-being and good health.