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The ITP Support Association is grateful to Professor Adrian Newland and Dr John Grainger for their assistance in the preparation of this page.
Further reading: The ITP Support Association’s core booklet Know About ITP (free to members and sent out with the initial information pack) explains about ITP, its management, treatment options, general outlook, ITP in pregnancy, and referral and second opinion on the NHS.
Immune Thrombocytopenia (An autoimmune disorder (Immune) causing a shortage of small cells in the blood known as platelets (Thrombocytopenic) Formerly known as Idiopathic (of unknown origin) Thrombocytopenic Purpura (bruising).
A disorder in which the body’s immune system destroys platelets in the blood. A normal platelet count is between 150- 400 (UK measurement). People with ITP can have a platelet count under 5.
Immune thrombocytopenia (formerly known as idiopathic thrombocytopenic purpura) is a medical term for an autoimmune disorder (immune) causing a shortage of platelets (thrombocytopenia) and bruising (purpura).
For most people there is no known cause, but for others, particularly children it can follow a simple viral infection such as a cold or sore throat. It has a slightly higher incidence in pregnancy and can also arise whilst taking some medications or following vaccinations. ITP can occur as a secondary condition to certain other illnesses.
ITP is an autoimmune disease in which the immune system mistakes the platelets as being foreign and destroys them. It can follow a virus, vaccination or certain medications, but for most people the cause is unknown.
ITP that arises suddenly is known as acute ITP, if the platelet count remains low after 3 months it will be called persistent ITP, and if the platelet count has not returned to normal after 12 months it will be called chronic ITP. The severity of the condition is determined by adding the names severe or mild. Thus, for example, someone with chronic severe ITP would have had a troublesome condition with a very low platelet count for over a year.
No, unlike autoimmune diseases such as rheumatoid arthritis and m ultiple s clerosis it is not a progressive disease, but the risk of bleeding is present from the day the disease develops.
This rare condition can arise in anyone at any age, but acute ITP (less than three months duration) is more common in children , and persistent ITP (up to 1 year) and chronic ITP (often lasting for years or lifelong) is more common in adults.
ITP is usually diagnosed by a blood test showing that only the platelet count is low, and the platelets, red blood cells and white blood cells all look normal. A bone marrow biopsy may be taken at a later stage if the ITP continues, in which a small sample of bone marrow will be taken under local anaesthetic and examined under the microscope. Additional blood tests may be taken at this time to exclude rare clotting or immune diseases that can mimic ITP. If the bone marrow looks normal, with the usual or higher number of platelet parent cells (megakaryocytes) and other blood tests are normal then the doctor will diagnose chronic ITP.
There are only 3000 – 4000 ITP patients in the UK at any one time. Most general hospitals will only be seeing one or two adults and children with ITP.
At present doctors are unable to predict who will have an episode of serious bleeding. Many people with ITP have few problems, whilst others have a more difficult condition which needs aggressive treatment. People with a count over 50 are much less likely to have severe symptoms than those whose count is in single figures. It is extremely rare for ITP to cause a fatal haemorrhage.
Treatments, which are currently aimed at suppressing or confusing the immune system, all have their dangers and drawbacks, only temporarily raise the platelet count, but do not cure the disease.
Some adults with mild ITP may not need any treatment, but will be monitored with occasional blood tests. There is no cure for ITP and treatment is used to raise the platelet count to counteract symptoms.
First line treatments include prednisolone and intravenous immunoglobulin (under various brand names).
Second line treatments includesplenectomy, azathioprine, cyclosporin, cyclophosphamide, vinca alkaloids, danazol, dapsone, rituximab, eltrombopag, romiplostim and mycophenlate mofetil.
Tranexamic acid may be used for dental extractions. Hormone preparations and/or tranexamic acid may be prescribed to women having heavy periods.
Platelet transfusions may be used to stem active bleeding or during surgery, but they are ineffective as a treatment for ITP as the immune system destroys transfused platelets as quickly as the body’s own platelets.
Hæmophilia is inherited and permanent, ITP is not inherited, and platelet counts can return to normal. Hæmophilia patients are deficient in one of the 12 factors which act together to form a blood clot. ITP patients are short of platelets which work independently as the initial plug to stop blood leakage. Platelet infusions are only used in emergencies as transfused platelets, like the patient’s own platelets, are destroyed by their immune system in a matter of hours.
No it is not caught and can not be passed on.
There are three types of blood cell which are all formed in the bone marrow; red cells, white cells and platelets. Platelets, which are small and sticky and circulate in the bloodstream provide the inital plug to stop bruising and bleeding after an injury, and stop blood leaking from capilleries. A blood sample is taken to measure the circulating platelets, and a normal platelet count is between 150 to 400 (x 109/l). Anyone with a count less than 100 (known as 100,000 in the US) would be considered thrombocytopenic (ie. short of platelets). Many people with ITP have a platelet count in single figures, and on rare occasions there are not enough circulating platelets to be counted, thus the count is given as 0. The number of platelets circulating in our bodies fluctuate all the time, and thus no two consecutive platelet counts are likely to be exactly the same either in a healthy person or in an ITP sufferer.
In the UK about 3,000 to 4,000 of the population have ITP at any one time, and it is not more prevalent in any particular racial or ethnic group.
Some people with ITP, especially those with a count over 50, may have no symptoms at all, and their ITP only noticed during a routine blood test. Even people with very low counts, can sometimes have few symptoms.
Common symptoms are:
petechiae (pinprick rash of blood spots)
black mouth blisters
Rare symptoms are:
blood in the eyes
bleeding from the ears
blood in the urine
bleeding from the gut
bleed into the brain
You should contact the hospital in the following circumstances:
A prolonged (over 30 minutes) nosebleed which will not stop despite pinching the nose
Prolonged gum bleeding
Blood in the faeces or urine
A heavy blow to the head
Persistent or severe headache with loss of vision, vomiting or drowsiness
Very heavy periods (eg. lasting 8 -10 days, changing more than 9 tampons/pads per day, changing protection during the night, passing clots on 2 consecutive days)