Resources
Clotting Disorders

Bleeding and clotting disorders are oftentimes interrelated with one another, making it a challenge to discuss them independently. Even though quite complex in nature, clotting disorders can by described as either an increased risk of thrombosis (clotting) or a defect of the anticoagulant mechanisms, which inevitably causes a disruption in the hemodynamic state of the individual. Clinical features can be any of the following: idiopathic, genetic, recurrent thrombosis, and thrombosis in an unusual site. In general, anticoagulant therapy is the management of choice, but it is important to consult a physician before starting any type of therapy.

Several types of clotting disorders will be featured, but refer to the resources provided to find out about additional types of clotting disorders.

• Idiopathic Thrombocytopenic Purpura
• Thrombotic Thrombocytopenic Purpura

Immune Thrombocytopenic Purpura

What is it?
Immune Thrombocytopenic Purpura (ITP) is a disorder of the blood.

Immune refers to the immune system's involvement in this disorder. Antibodies, part of the body's immunologic defense against infection, attach to blood platelet, cells that help stop bleeding, and cause their destruction. Thrombocytopenia refers to decrease in blood platelet. Purpura refers to the purplish-looking areas of the skin and mucous membranes (such as the lining of the mouth) where bleeding has occurred as a result of decreased platelet.

Some cases of ITP are caused by drugs, and others are associated with infection, pregnancy, or immune disorders such as systemic lupus erythematosus. About half of all cases are classified as "idiopathic," meaning the cause is unknown.

What are the symptoms of ITP?
The main symptom is bleeding which can include bruising ("ecchymosis") and tiny red dots on the skin or mucous membranes ("petechiae"). In some instances bleeding from the nose, gums, digestive or urinary tracts may also occur. Rarely, bleeding
within the brain occurs.

How is ITP diagnosed?
The physician will take a medical history and perform a thorough physical examination. A careful review of medications the patient is taking is important because some drugs can be associated with thrombocytopenia. A complete blood count will be done. A low platelet count will establish thrombocytopenia as the cause of purpura. Often the next procedure is a bone marrow examination to verify that there are adequate platelet-forming cells (megakaryocyte) in the marrow and to rule out other diseases such as metastatic cancer (cancer that has spread to the bone marrow) and leukemia cancer of the blood cells themselves). Another blood sample may be drawn to check for other conditions sometimes associated with thrombocytopenia such as lupus and infection.

Acute and Chronic Form of Thrombocytopenic Purpura
Acute (temporary) thrombocytopenic purpura is most commonly seen in young children. Boys and girls are equally affected. Symptoms often, but do not necessarily, follow a viral infection. About 85 percent of children recover within 1 year and the problem doesn't return.

Thrombocytopenic purpura is considered chronic when it has lasted more than 6 months. The onset of illness may be at any age. Adults more often have the chronic disorder and females are affected two to three times more than males. The onset of illness may be at any age.

How is ITP treated?
If the doctor thinks a drug is the cause of the thrombocytopenia, standard treatment involves discontinuing the drug's use. Infection, if present, is treated vigorously since control of the infection may result in a return of the platelet count to normal.

The treatment of idiopathic thrombocytopenic purpura is determined by the severity of the symptoms. In some cases, no therapy is needed. In most cases, drugs that alter the immune system's attack on the platelet are prescribed. These include corticosteroids (i.e., prednisone) and/or intravenous infusions of immune globulin. Another treatment that usually results in an increased number of platelet is removal of the spleen, the organ that destroys antibody-coated platelet. Other drugs such as vincristine, azathioprine (Imuran), Danazol, cyclophosphamide, and cyclosporine are prescribed for patients only in the severe case where other treatments have not shown benefit since these drugs have potentially harmful side effects.

Excepts in certain situation (e.g., internal bleeding and preparation for surgery), platelet transfusion usually are not beneficial and, therefore, are seldom performed. Because all therapies can have risks, it is important that overtreatment (treatment based solely on platelet counts and not on symptoms) be avoided. In some instances lifestyle adjustments may be helpful for prevention of bleeding due to injury. These would include use of protective gear such as helmets and avoidance of contact sports in symptomatic patients or when platelet counts are less than 50,000. Otherwise, patients usually can carry on normal activities, but final decisions about activity should be made in consultation with the patient's hematologist.

Where can I obtain further information on ITP?
Blood specialists (hematologist) are experts in the diagnosis and treatment of these disorders. These doctors practice in most mid-and large-size cities. A majority of medical centers have hematology divisions in their medicine or pediatrics departments, and patients who need evaluation, treatment, or information can often be referred there.

Source: National Women's Health Information Center

Additional resources:

• National Heart, Lung, and Blood Institute
• Excessive Blood Clotting (Hypercoagulation)
• THROMBOLYSIS, THROMBOSIS, THROMBUS AND EMBOLUS (American Heart Associaton)
  
  

Thrombotic Thrombocytopenic Purpura

What is thrombotic thrombocytopenic purpura?
Thrombotic Thrombocytopenic Purpura (TTP) is a disorder of the blood.

Thrombotic refers to blood clots that form, obstructing the small blood vessels in the body which can cause damage to the kidneys, heart, and brain. Subsequently, the occlusion causes fragmentation of red blood cells and hemolysis (destruction of red blood cells). Thrombocytopenia refers to decrease in blood platelets. Purpura refers to the purplish- looking areas of the skin and mucous membranes (such as the lining of the mouth) where bleeding has occurred as a result of decreased platelet.

The exact cause of TTP is unknown. Some cases of TTP are associated with the use of estrogen or by pregnancy. It is also associated with HIV, cancer, bacterial infection, vasculitis, bone marrow transplantation, and drugs.

What are the symptoms of TTP?
The main symptom is bleeding which can include bruising ("ecchymosis") and tiny red dots on the skin or mucous membranes ("petechiae"). In some instances bleeding from the nose, gums, digestive or urinary tracts may also occur. Other symptoms include fever, rash, headache, fatigue, abdominal pain, altered mental status, slight or partial paralysis, seizures, and jaundice (yellowish color to skin).

How is TTP diagnosed?
The physician will take a medical history and perform a thorough physical examination. A careful review of medications the patient is taking is important because some drugs can be associated with thrombocytopenia. A complete blood count will be done. A low platelet count will establish thrombocytopenia as the cause of purpura. A peripheral blood smear may be done which may show fragmented red blood cells consistent with hemolysis. Urinalyisis will confirm presence of protein and blood. Mucous membrane biopsy may show clots in small blood vessels.

How is TTP treated?
If the doctor thinks a drug is the cause of the thrombocytopenia, standard treatment involves discontinuing the drug's use. Plasmapheresis is the standard of care to remove unwanted substances from the blood. Prednisone may be used to reduce the destruction of platelets. Another treatment that usually results in an increased number of platelets is removal of the spleen, the organ that destroys antibody-coated platelet.

Where can I obtain further information on TTP?
Blood specialists (hematologist) are experts in the diagnosis and treatment of these disorders. These doctors practice in most mid-and large-size cities. A majority of medical centers have hematology divisions in their medicine or pediatrics departments, and patients who need evaluation, treatment, or information can often be referred there.

Source: National Organization for Rare Disorders

Additional resources

• Thrombolytic Professional Forum and Resource Center