Idiopathic Thrombocytopaenic Purpura

What Is Idiopathic Thrombocytopaenic Purpura?

Commonly referred to as ITP, idiopathic thrombocytopaenic purpura is a blood disorder which prevents blood from clotting properly. ITP occurs as a result of decreased levels of platelets (types of blood cell involved in the clotting process which help to stem bleeding). People who have ITP regularly suffer with purple bruises, which are caused by the tiny blood vessels underneath the skin bleeding.

The terms idiopathic, purpura and thrombocytopaenic mean:

Idiopathic: the reason for the condition is unknown.
Purpura: this refers to the purple bruises, which develop as a result of bleeding underneath the skin’s surface. Purple patches can also emerge on mucus membranes inside the mouth. The skin patches of purple often present as tiny dots, are most commonly visible on the legs and are known as petechiae (petechiae often resemble a rash).
Thrombocytopaenic: this means that there is a drop in the number or volume of platelets found in the blood.

Effects of ITP

People who have ITP often experience the following symptoms:

Nose bleeds.
Bleeding gums.
Heavy bleeding during periods.
Symptomatic bleeding in the brain (this is a rare occurrence).
Bleeding that is difficult to stem.

ITP is generally considered to be an autoimmune disease. These occur when the immune system mistakes something harmless for a threat and starts to attack healthy tissues. Many cases of ITP are often known as immune thrombocytopaenic purpura.

How do platelets work?

Platelets, also known as thrombocytes, are tiny blood cells that play an important role in the blood clotting process. Platelets are produced by the bone marrow, circulate in the blood stream and stem bleeding by collecting together.

Forms of ITP

Two forms of ITP are apparent. They are acute and chronic:

Acute ITP: acute ITP is a short-term condition, which doesn’t last beyond 6 months. This form of ITP is common in children and usually occurs following a viral infection. Acute ITP usually subsides without the need for treatment, and is diagnosed more often than chronic ITP.
Chronic ITP: chronic ITP is a long-term condition and lasts longer than 6 months. ITP is most common amongst adults but it can affect teenagers and children on rare occasions. Chronic ITP is between two and three times more common in women than men. In moderate cases treatment or procedures are often not required. Treatment options for more severe cases depend on the platelet volume and the severity of symptoms.

Other terms for idiopathic thrombocytopaenic purpura

Autoimmune thrombocytopaenic purpura
Immune thrombocytopaenic purpura.

Causes of idiopathic thrombocytopaenic purpura

In people who have ITP the body’s immune system mistakes the platelets for a harmful threat and starts to produce antibodies to attack the platelets. This causes the amount of platelets located in the bloodstream to drop. The antibodies attach to the platelets and are then removed from the body by the spleen, which is located in the upper abdomen.

The immune system is usually responsible for attacking threats to prevent illnesses and infections, such as viruses and bacteria. But with autoimmune conditions the system mistakes healthy tissue for threats and starts to effectively attack the body. There is no definitive reason for why the body’s immune system cannot distinguish between the harmless and the harmful, and it is unknown why the body attacks the platelets.

Children who develop acute ITP have often recently had a viral infection and it is therefore believed that the virus may trigger the abnormal immune response. In adults there is no evidence to suggest a link between ITP and infections.

Who is most at risk of developing idiopathic thrombocytopaenic purpura?

Adults and children can both develop ITP. Acute ITP is more common in children, while chronic ITP is more common in adults.
Children often develop ITP following a viral infection.
It is rare for adults to develop acute ITP.
Females are between two and three times more probable to suffer from chronic ITP than men.
ITP is not contagious, which means that it cannot be passed from person to person.

Signs of idiopathic thrombocytopaenic purpura

The signs of idiopathic thrombocytopaenic purpura are caused by bleeding, due to the reduced quantity of platelets in the body. Symptoms include:

Bruising (purpura): this causes visible purple skin patches and in the mucus membranes. Bruising is usually caused by contact with an object or surface, but for those with ITP bruises may appear with no known cause.
Petechiae: these are small red or purple spots, which tend to emerge on legs. The spots often resemble a skin rash and are caused by bleeding underneath the skin.
Difficulty stopping bleeding.
Bleeding gums.
Heavy periods.
Nose bleeds.
Blood found in the urine.
Blood found in the faeces.
Bleeding found within the brain (this is a rare occurrence).

A low level of platelets increases the danger of bleeding, but it does not contribute to any additional symptoms, such as fatigue or pain.

How can you diagnose idiopathic thrombocytopaenic purpura?

In order to diagnose ITP doctors carry out a physical examination, order blood tests and take a full medical history. A medical history will include information concerning any underlying conditions you have, any conditions you have had in the past, your symptoms and any medication you are currently taking or have taken in the past.

Your doctor will carry out a physical examination to look for symptoms of ITP. During the examination they will be looking out for:

Bruising.
Petechiae.
Indicators of bleeding.
Signs of infection.

The GP may also carry out blood tests before they confirm a diagnosis. Blood tests will include:

Complete blood count (also known as a full blood count): this test measures the levels of all types of blood cells (white blood cells, red blood cells and platelets).
Blood smear test: this involves analysing a tiny sample of blood under a microscope to examine the platelets in detail. The blood smear test is vital to check that the platelet volume or count is accurate.

If the test results indicate that a patient has a low level of platelets, additional tests will usually be carried out to confirm a diagnosis and determine the reasons behind the reduced platelet count, which may include bone marrow tests.

Individuals with ITP usually have normal levels of red and white blood cells.

Low platelet quantity is often the result of the body destroying platelets or not producing enough platelets. In cases of ITP the platelet quantity or count is lower than normal, since the body damages the platelets before the bone marrow is able to produce new platelets.

In some cases individuals with ITP do not display indicators of bleeding. If this is the case condition may only be diagnosed once tests have been conducted for another condition and happen to show that the quantity of platelets in the blood is low. Once other causes have been checked then ITP will usually be found to be the cause.

How do you treat idiopathic thrombocytopaenic purpura?

The course of treatment will depend on platelet numbers and the severity of symptoms. In some mild cases no treatment is required.

Treatment for adults with ITP

Adults with mild cases of ITP sometimes do not require treatment. However, their platelet count and symptoms will be monitored closely. In cases where bleeding symptoms are more severe and the platelet count is low, medication is usually prescribed.

Treatment for children with ITP

In the vast majority of cases children are affected by acute ITP, which tends to subside fairly quickly within weeks without treatment. Treatment is recommended for children who have bleeding symptoms, but in milder cases no treatment is normally required. All children will be monitored closely regardless of whether or not they are given a formal course of treatment.

Medicines

Medication is usually the first course of treatment for adults and children with ITP. Corticosteroids (normally prednisone) are usually used first, as they help to increase the platelet count by reducing the action of the body’s immune system. Steroids can have several complications and side-effects, however, and a number of people can relapse after they have stopped taking steroids.

Some medicines, including anti-D immunoglobulin and immune globulin, are administered intravenously (this means the medicine is inserted by means of a needle into a vein).

If corticosteroids are not effective other types of medication may be prescribed. This usually occurs following a splenectomy (a procedure to remove the spleen).

Taking out the spleen (splenectomy)

In some cases, where medication has been unsuccessful, it may be necessary to remove the spleen by means of a procedure called a splenectomy. In most cases this is carried out on adults with chronic ITP who have not reacted to medicines. Taking out the spleen helps to prevent damage to platelets, though it can increase the risk of infection. It is therefore highly recommended for people who have had their spleen removed to look out for symptoms of infection and seek medical attention immediately if they discover any.

Other treatments

Platelet transfusion: some individuals with severe ITP may be required to go into hospital to have a platelet transplant; and platelets can be transplanted prior to surgery.
Treatment for infections: in some cases infections can reduce the volume of platelets, meaning that treatment for the infection will cause the platelet count to increase to normal levels.
Stopping medicines: some medicines can reduce the platelet count. So if a patient is using medication that could decrease the platelet volume or lead to bleeding, stopping taking it could help to increase platelet numbers.

Living with the condition

If you suffer with ITP you should follow this advice:

Avoid taking medication that can disrupt platelet function and heighten the danger of bleeding (for example, aspirin and ibuprofen).
Avoid activities or environments where injuries and accidents are likely, which reduces the risk of bruising and bleeding.
Look for a physician who has experience in treating patients with ITP.
If your spleen has been removed look out for symptoms of infection (including high temperature) and see your doctor as soon as possible if you think you have an infection.

ITP and pregnancy

Pregnant females who have ITP generally give birth to healthy babies without any problems. However, the baby may be born with a decreased platelet count or develop small platelet numbers shortly after birth. The platelet volume will resume a normal, stable level shortly after, without any need for treatment. However, treatment can be recommended for babies who have low platelet numbers to speed up the process of platelet numbers returning to normal.

Treatment for pregnant females suffering with ITP is dependent on the severity of the symptoms and the platelet count. Where treatment is required doctors will consider the possible effects upon the unborn child and choose the treatment type that is safest for both mother and baby.

Women who have a mild form of ITP may be able to continue their pregnancy without having any treatment, but their condition will be monitored closely.

ITP key points

It is a bleeding condition that causes bruising and makes it difficult to stem bleeding.
ITP prevents the blood from clotting normally.
A low level of platelets within the blood causes ITP. Platelets are blood cells that are involved in the clotting process.
ITP causes a low volume or low numbers of platelets due to the immune system’s destruction of the platelets, which it mistakes for a harmful threat. It is unknown what causes this immune system response.
Individuals with ITP experience symptoms including bruising and minute red dots, which are caused by bleeding underneath the skin.
The condition can also cause bleeding gums, blood in the urine, nose bleeds and heavy menstrual bleeding.
ITP can have an effect on males and females of any age.
Two forms of ITP are evident: acute (short-term) and chronic (long-term). Acute ITP is more common in children, while chronic ITP is more common in adults.
In children acute ITP often develops following a viral infection. Most children recover after a short period of time without any need for treatment.
Adults usually develop chronic ITP.
ITP isn’t the only condition that causes low platelet count, so a series of tests are performed to confirm diagnosis.
Treatment for ITP is based on the severity of symptoms and the platelet count. Medication (usually corticosteroids) is used first in the majority of patients with moderate or severe symptoms.
In some cases the spleen is removed in a procedure called a splenectomy. This is usually carried out when an adult patient has severe symptoms and they have not reacted to medication.