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Immune Thrombocytopenic Purpura (ITP)


Immune thrombocytopenic purpura (ITP) is an autoimmune disease or a disorder of the blood. It is an acquired illness (you are not born with it). ITP occurs when antibodies from the immune system attack the body’s platelets, which are the cells that help the blood to clot.

When antibodies attack the platelets, the number of platelets in the body is reduced (thrombocytopenic). When the platelet count is very low, patients may be at a higher risk for bleeding (purpura). This bleeding may occur in response to an injury, a surgery, a procedure, or suddenly with no obvious cause.


Types of ITP
  • Acute – this type is most common in children, particularly between the ages of two and four. ITP often occurs after a viral infection. Symptoms usually disappear within a year, and do not recur.
  • Chronic – this type is most common in adults, particularly young women. ITP is considered chronic if it lasts more than six months.

Causes

The exact cause of ITP is not always known. ITP may be caused by
  • Autoimmune or inflammatory condition such as lupus or rheumatoid arthritis
  • Use of certain medications
  • ITP caused by antibodies to platelets that lead to opsonization and destruction of platelets in the spleen.
  • Infection
  • Pregnancy
  • Immune disorders
  • Systemic lupus erythematosus
  • Viral infections in children
  • Idiopathic (unknown) causes

Risk Factors

A risk factor is something that increases your chances of getting a disease or condition. Risk factors for ITP include
  • Age: childhood
  • Sex: in adults, women are more likely than men to develop ITP
  • HIV infection
  • Pre-eclampsia
  • Cardiopulmonary bypass surgery
  • Presence of other autoantibodies in the blood
  • Infection
Symptoms and signs

The main symptom of ITP is bleeding. This may result in easy bruising or tiny red dots on the skin or mucous membranes. Occasionally, bleeding will occur in the nose, gums, or digestive or urinary tracts. Women may experience abnormal heavy menstrual bleeding. In rare cases, severe spontaneous bleeding (bleeding that happens without any injury or known cause) may occur from the gastrointestinal tract or into the brain.

Atypical symptoms include fever and bone pain, and atypical signs include leukocytosis, leukopenia, anemia, hepatosplenomegaly or lymphadenopathy. The major confounding diagnosis is leukemia. Any patient being worked up for ITP should trigger thoughts of "could this be leukemia"..?


Is it very dangerous to have a low platelet count

Although the numbers of platelets are reduced, the bone marrow is working hard supplying new platelets to replace the ones being removed by the immune system and the platelets that are present work very well. Although the symptoms described above can be alarming at first they are usually not dangerous and the safest thing to do once the correct diagnosis has been made is to just wait for the platelet count to recover on its own.


One important and serious problem that can occur very rarely is bleeding inside the head. This is called "intra cranial haemorrhage". It is important to contact your hospital if your child has a head injury whilst their platelets are low so that they can be checked over and observed on a ward if necessary. It is also important to contact a doctor if you feel your child is not themselves, with drowsiness, vomiting weakness or fits.

However this is very unusual, but it makes sense to be alert for these symptoms and to avoid activities where there is an obvious danger of a bad head injury until the platelet count begins to recover. Depending on the age of your child, these might include climbing frames, rollerblading, horse riding, contact sports, and so on. Individual activities can be discussed with your doctor.


Diagnosis

The diagnosis of immune thrombocytopenic purpura remains one of exclusion (diagnosis is made only after ruling out other possible causes for the decrease in platelets. This is what doctors call a diagnosis of exclusion).

Also secondary forms of the disease occur in association with systemic lupus erythematosus, the antiphospholipid syndrome, immunodeficiency states (IgA deficiency and common variable hypogammaglobulinemia), lymphoproliferative disorders (chronic lymphocytic leukemia, large granular lymphocytic leukemia, and lymphoma), infection with human immunodeficiency virus and hepatitis C virus, and therapy with drugs such as heparin and quinidine.

There is no diagnostic test to absolutely say a patient has ITP. Your doctor will ask about your symptoms and medical history, including medication use, and perform a physical exam. Tests may include:
  • Blood tests
  • Urine tests
  • Bone marrow exam
  • Tests to look for antibodies against platelets
Treatment

Treatment will depend on the cause of ITP and the severity of the symptoms. Severe bleeding problems do not usually happen unless a person's platelet count is less than 20,000. If there are no bleeding symptoms, treatment is not needed unless the platelet count is less than 20,000. But if a person does have bleeding problems, treatment may be started even though the platelet count is higher than 20,000.

Treatments for chronic ITP include steroids (prednisone, dexamethasone), dapsone, vincristine, 6 mercaptopurine, cytoxan and rituximab.

In general many treatments may be used in ITP. Most patients will be treated with prednisone, which is a steroid medication, as a part of the initial therapy. There are many other treatments which may be used to treat a patient with ITP. These include:
  • Removal of the spleen (splenectomy)
  • An IV (intravenous) medication that treats problems with your immune system (Immunoglobulin)
  • Other medications that suppress your immune system
You should discuss these treatment options in detail with your physician to see which may be best for your particular situation.

And because children and adults usually develop different types of ITP, their treatments may be different.


Treatment for Children

Usually, children with ITP recover without any treatment. Your doctor will monitor your child’s recovery and treat bleeding symptoms. In certain cases, a doctor may recommend increasing the platelet count with prednisone pills or intravenous infusions of gamma globulin.


Treatment for Adults

First, your doctor will check your medications to determine if any are causing ITP. If so, that medication will be discontinued. Other treatments include:
  • Treatment of infection, if present
  • Medications that alter the immune system’s attack on platelets, such as:
  • Corticosteroids (e.g., prednisone)
  • Intravenous infusion of gamma globulin
  • Danazol (Danocrine)
  • Azathioprine (Imuran)
  • Cyclophosphamide (Cytoxan, Neosar)
  • Vincristine (Oncovin, Vincasar PFS, Vincrex)
  • Vinblastine (Velban)
  • Interferon
  • Surgery to remove the spleen
  • Filtering of the blood to remove harmful antibodies that are destroying platelets

Emergency management for critical bleeding

Both children and adults may require platelet or other blood transfusions if there has been serious blood loss.

Life-threatening bleeding requires immediate administration of platelet transfusions, intravenous methylprednisolone (1000 mg), and intravenous immunoglobulin. Although platelet count increments may be small and transient after a platelet transfusion, platelet counts do increase in many patients and most patients have a hemostatic benefit. For continued bleeding, intravenous factor VIIa may be effective


Prevention

There are no guidelines for the prevention of ITP.However, if you develop ITP, it is recommended that you take certain precautions to prevent ITP from becoming worse. These precautions include:
  • Use protective gear (such as helmets) when participating in risky activities such as bicycling
  • Avoid contact sports
  • Avoid use of aspirin and ibuprofen, as these medications slow blood clotting time

Regular Blood donors face a reduced risk of heart problems.
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