Immune thrombocytopenia

This information is for people with immune thrombocytopenia (ITP) who are registered with our ITP centre at Norfolk & Norwich University Hospital. If you are not a patient here but need help with ITP, please contact your own centre or GP.

A leaflet version of this webpage is available to download here

What are platelets?

Platelets are one of the three types of blood cell, along with red and white blood cells. Platelets are small and sticky and their job is to prevent bruising and stop bleeding after an injury. Platelets, like red and white blood cells, are formed in the bone marrow. A rough idea of how many platelets are circulating in the bloodstream (platelet count) can be made from a sample of blood. The normal platelet count is between 150 and 400 x 109/L. In many cases of ITP, the platelet count is less than 30 x 109/l at presentation. A low platelet count is called “thrombocytopenia”.

What is immune thrombocytopenia (ITP)?

ITP is a medical term for a condition in which there is bruising or bleeding because there are fewer platelets in the blood than usual (thrombocytopenia) and is usually caused by something going wrong with the immune system (the body’s defence against infection). ITP is not a genetic/inherited condition.

How common is ITP and who does it affect?

Approximately 4 in every 100,000 people develop ITP every year. It can occur at any age but is slightly more common in women than men.

What causes ITP?

ITP is an autoimmune disease. In some cases, this is due to a self-reacting antibody binding to the surface of the platelets, causing both to be removed by cells in the spleen and elsewhere. However, other immune abnormalities have been found in some patients with ITP that affect lymphocytes (a type of white cell) or megakaryocytes (the precursor cells of platelets). These differences may help explain why patients vary in their response to treatment. Why some people develop this “autoimmune” process and others do not is not fully understood. In some individuals, a viral infection may act as an environmental trigger to the process and your doctors may test for triggers such as infection when you are first assessed.

Symptoms of ITP

If the platelet count is in the range 50–100 × 109/l there is usually no bleeding or bruising. If the platelet count falls below 50 × 109/l, some bruising may occur, and below 20 ×109/l, bruising and petechiae (pinpoint blood spots under the skin) are more likely. Bleeding may occur from mucous membranes such as the nose and gums while female patients may suffer with heavy periods. The most serious bleeding tends to occur in patients with a platelet count <10 × 109/l. However, with ITP most patients with platelets less than 10 × 109/l still have no major bleeding problems.

As with any medical condition, ITP may affect your quality of life. For example, about a third of those with ITP report fatigue symptoms. You may be worried about how bleeding might affect work and social activities. For most people, the impact of ITP on their quality of life seems to reduce after the first year, and in those with a good response to treatment. The impact of ITP will vary from person to person and the reasons for symptoms may also differ, so discuss any concerns you have with the doctor who is managing your ITP.

Diagnosis of ITP

There is no single blood test that can prove you have ITP, and it remains a diagnosis of exclusion based on history, examination and the results of your initial tests. Investigations are mainly blood tests and are focused on looking for underlying causes of low platelets (other causes for low platelets include vitamin deficiencies, medication, or a bone marrow abnormality). If there are any unusual features on initial tests, we may recommend a bone marrow examination, which is a day unit procedure where we take a small sample of marrow from the pelvic bone under local anaesthetic.

Treatment and outlook for adults with ITP

In most cases, ITP presenting in adulthood is a chronic condition. The goal of treatment is to maintain a platelet count that is safe while minimising treatment side effects and maintaining a good quality of life. For this reason, treatment is often not required when the platelet count is above 20 × 109/l unless there is a need to cover a period of increased bleeding risk, such as surgery or dental extraction. However, the decision to treat is individualised and will also depend on your symptoms, lifestyle and medical history.

First line treatment is typically with tablet steroids such as prednisolone. About 70-80% of individuals will respond to steroids and initial treatment will usually be for 6-8 weeks, although the dose will be reduced during that time, rather than stopped suddenly. Steroids can have side effects. You may not have side effects but these can include gastric irritation (we may recommend an additional tablet to protect the lining of the stomach), mood or sleep disruption, greater risk of infection, fluid retention, increased appetite, increased blood sugar or blood pressure, muscle weakness and thinning of the bones. For some individuals, we may also recommend additional treatment to protect the bones while on steroids.

For those who do not respond to first line treatment, or who lose their initial response (up to 80% of responding patients will ultimately find that their platelets subsequently fall), there are a number of treatment options. Some of those that may be discussed are:

• Oral immunosuppression (e.g. mycophenolate, azathioprine)
• Rituximab
• Thrombopoietin receptor agonists (e.g. eltrombopag, romiplostim)
• Intravenous immunoglobulin
• Splenectomy

Tranexamic acid is a medication that helps blood clots to last longer once they have formed. Tranexamic acid is not a treatment for ITP but can be useful if you have bleeding while your platelet count is low. It is a tablet taken 3 times a day. It should not be taken if you have blood in your urine. It can sometimes cause indigestion, which may improve if you take a lower dose.

Platelet transfusions can be useful as an emergency treatment for severe bleeding, as they can help you form a clot. However they are not useful for long term prevention of bleeding because they will only last for minutes to hours before being destroyed by your immune system.

What else can I do?

It would be sensible to avoid sports where there is a risk of head injury whilst the platelet count is below 50 × 10^9/l. With a platelet count between 50 and 100 × 10^9/l there will still be more bruising so consider the use of shin pads etc. For further details, discuss with your consultant. There may be times when taking a holiday abroad is better avoided; discuss this with your doctor. It may be harder to get insurance. A list of recommended insurance companies can be obtained from the ITP Support Association (details below).

You should avoid drugs like aspirin and non-steroidal anti-inflammatory drugs (e.g. ibuprofen, diclofenac) when your platelets are below 50 × 10^9/l because of a greater risk that gastric irritation could lead to bleeding. Also discuss any planned surgery or dental procedure with your haematologist (as well as informing the dentist or surgeon) so that a plan can be made.

When to seek help

• If you have minor bleeding symptoms such as nose bleeds or bleeding in the mouth please ask your GP to carry out an urgent full blood count, or contact the haematology department. A purple rash called purpura (often on the lower legs) which does not fade when you press it may be a sign of a low platelet count.

• If you have a prolonged episode of gum bleeding (over 30 minutes) or nose bleeds that will not stop despite pinching the nose, contact the hospital.

• If you vomit blood or have blood in the faeces or black sticky stools or other major bleeding you may need urgent medical help; please contact the haematology department or attend the Emergency Department of the nearest hospital.

• Also contact the hospital if you develop a severe headache when you have low platelets. Bleeding into the brain (intra-cranial bleeding) is a very rare but serious complication of ITP.

Contacting the haematology unit

Emergency contact number: 01603 646753
During the daytime Monday to Friday, the Haematology Clinical Nurse Specialists will answer your call. Outside these times, a nurse on the Haematology ward will speak to you.

Support group in the UK for people with ITP

UK ITP Support Association

Clinics for people with ITP at Norfolk & Norwich University Hospital

Patients will ITP who need to attend will usually be seen in clinic on Monday mornings. You will book in with the receptionist who will check your details. You will usually have a blood sample taken by the phlebotomist (the person who takes you blood samples). We may also ask you to have a capillary blood glucose fingerprick test if you are taking steroids.

A number of our patients can have remote consultations (by video or telephone) if they do not need to attend clinic, with a blood test arranged in advance. This can be done at their GP surgery or a hospital phlebotomy unit.

When the consultation is finished you will be given a booking sheet to take to the receptionist who will book another appointment. You will usually receive a letter giving details of your next appointment.

The doctor will try to see you within 30 minutes of your arriving in clinic but this is not always possible. Sometimes we will have seriously ill patients in clinic whose consultations take more time than usual.

What should I do if I can’t come to clinic?

It is very important that you let us know if you cannot come to clinic. If you phone to let us know that you cannot come to clinic we can book someone else into your appointment slot. If you are unable to attend clinic please call on 01603 287866 between 8.30 am and 4pm.

Thanks to Leeds Teaching Hospital Haematology Department for permission to use content from their patient information leaflet