Immune Thrombocytopenia (ITP) is a disorder resulting in low levels of platelets in the blood (low platelet counts).^1-3 Platelets help the blood to clot. A normal platelet count is between 150,000 to 400,000 per mL of blood (this may be written as 150-400 x10⁹/L),⁴ whilst an individual with ITP will have less than 100,000 per mL.³ This is due to both the body’s immune system attacking and destroying the platelets, and the body being unable to produce enough platelets to compensate for the increased destruction (impaired platelet production). In ITP, the low levels of platelets stop the blood from clotting properly, which increases the risk of bleeding and can lead to a range of symptoms.

ITP may be newly diagnosed (within first 3 months of diagnosis), persistent (between 3 to 12 months after diagnosis) or it can become chronic (long-term), where it lasts for twelve months or more and may require ongoing treatment.⁵ ITP can affect both children and adults; however, it is more common for adults to progress to chronic ITP whilst in most cases, children experience self-limiting ITP, which can often resolve without treatment. ^2,3

Synonyms: Autoimmune thrombocytopenic purpura; Idiopathic thrombocytopenia purpura; Idiopathic thrombocytopenic purpura; immune thrombocytopenia; ITP; primary thrombocytopenic purpura; thrombocytopenic purpura, autoimmune 

Universal rare disease classifications provide a common language for recording, reporting and monitoring diseases. Please visit the Rare Disease Classifications page for more information about these internationally recognised classifications.

ORPHA:3002 Immune thrombocytopenia

ICD-11: 3B64.10 Immune thrombocytopenic purpura

Symptoms of ITP may include:^1-4,6

• extreme tiredness (fatigue)
• bruises on skin
• nosebleeds or bleeding from the gums
• small red or purple pinpoint-sized dots (petechiae) or larger patches (purpura) on the skin that does not turn white when pressed (does not blanch)
• heavy menstrual periods
• blood in the urine (haematuria)

Severe bleeding such as gastrointestinal bleeding (gastrointestinal haemorrhage) or bleeding in the brain (cerebromeningeal bleeding) may occur but is rare.³

In some cases, ITP may not cause any symptoms. It can also be self-limiting, particularly in children, which means the symptoms can go away often without treatment.

Please speak to your medical team to learn more about the signs and symptoms of ITP.

Rare diseases are often serious and progressive, exhibiting a high degree of symptom complexity, leading to significant disability. Majority of the estimated two million Australians living with a rare disease meet the Australian Government’s definition for disability (in accordance to the Australian Public Service Commission and Australian Bureau of Statistics), and many experience severe and permanent disability impacts. If you or someone you care for is experiencing disability-related impacts from a rare condition, please speak with a health or disability professional for advice. Information about relevant disability support can be found at the RARE Portal’s Disability Support Information page.

In ITP, the body’s immune system produces antibodies that attack and destroy the platelet cells, leading to a low platelet count, coupled with impaired platelet production.^1,3 The cause of this immune reaction is not understood. It is thought that this may sometimes occur after a viral infection (such as a cold) as the immune system may become overactive during an infection and mistakenly start targeting its own cells.^2,4

ITP can also be caused by certain medications or can be due to other conditions (secondary ITP), for example, autoimmune diseases such as systemic lupus erythematosus, chronic viral infections such as hepatitis C virus and human immunodeficiency virus (HIV), primary immune deficiency and cancer.^3,7

ITP is not inherited and if there is a hereditary pattern, these is more suggestive of an alternative diagnosis, particularly the hereditary thrombocytopenia syndromes.

There is no specific test to diagnose ITP. As there are many possible reasons for a low platelet count, immune thrombocytopenia (ITP) is diagnosed by ruling out other causes of a low platelet count (diagnosis of exclusion). This may depend on age, sex and response to treatment.⁷ Personal medical history (including types of medications used), family medical history, physical examination, and blood tests (including blood film examination) may be used to rule out other conditions. Kidney and liver function tests may also be done – as kidney and liver function are not affected in ITP, abnormal test results would suggest that there are other conditions contributing to the low platelet count.⁷

Examples of conditions that should be ruled out are myelodysplastic syndromes (MDS), inherited thrombocytopenia’s, genetic immunodeficiency syndromes (typically common variable immunodeficiency), hypersplenism and liver disease.^3,7

Individuals with ITP may also be tested for hepatitis C virus and human immunodeficiency virus (HIV) as these can be secondary causes of ITP.⁷

Please speak to your medical team to learn more about the available pathways for the diagnosis of ITP.

Treatment of ITP may differ between individuals. There are specific consensus guidelines for management of adults and children (paediatric) in Australia.

In some cases, the condition may not require any treatment and can be managed by monitoring. For some individuals, particularly in children, the condition may be self-limiting, which means the symptoms may go away (resolve) without any treatment.^3,8

Treatment of ITP may depend on platelet count, bleeding risk, disease stage, age of individual, side effects of the treatment as well as other considerations.⁷ Whilst treatment is often aimed at increasing the platelet count to at least more than 20,000 per mL and to prevent serious bleeding, it has been recommended that patient’s quality of life and patients reported outcomes measures (PROMs) are taken in account to select the most suitable treatment strategy.⁹

Initial treatment options (first line treatment) may include use of medications such as corticosteroids, and occasionally intravenous immunoglobulin (IVIg), which is the infusion of plasma (liquid portion of blood) solution from healthy donors.^3,7,8 If the first line treatment is not effective enough, other therapies may be considered and used as second line treatment; this may include other types of medications, such as thrombopoietin receptor agonists (TPO-RAs) and biologics, and surgical removal of spleen (splenectomy) in adults as the spleen is where the platelets are destroyed. Combination therapies (using different types of treatment) may be used in some cases.

In cases where hepatitis C virus or human immunodeficiency virus (HIV) have been identified as the cause of ITP, the course of treatment is often different and involves treating the underlying virus.^7,9

Some individuals may go into remission, where symptoms may disappear completely or become very mild for a period of time.

Please speak to your medical team to learn more about the possible treatment or management options for your condition. Treatment will depend on an individual’s specific condition and symptoms. It is also important to stay connected to your medical team so that you can be made aware of any upcoming clinical trial opportunities. For many rare diseases, treatment options may be limited. Participation in a clinical trial may provide access to new or emerging therapies.

Healthcare professionals involved in the care of individuals with ITP may include general practitioners (GP), paediatricians, haematologists and immunologists.¹ The need for different healthcare professionals may change over a person’s lifetime and extend beyond those listed here.

Clinical care for rare diseases often involves a multidisciplinary team of medical, care and support professionals. Please note that the information provided here is as a guide and that RVA does not necessarily monitor or endorse specific clinics or health experts.

This may not be applicable to all rare diseases but for many, palliative care services may be relevant and useful. Palliative care services are available for people (adults, children and their families) living with a life-limiting illness and is not only for end-of-life care. It can also help at any stage of illness from diagnosis onwards, and will look different for different people. Palliative care services provide assistance, support, resources and tools to help people manage their illness and the symptoms, ease pain, and improve comfort and quality of life. If this is relevant to you and you wish to find out more information about palliative care and how it can help you, please visit:

• Australian Institute of Health and Welfare: Palliative care Overview
• Australian Government Department of Health, Disability and Ageing: Palliative care
• Palliative Care Australia

• Consensus guidelines for the management of adult immune thrombocytopenia in Australia and New Zealand (2021); includes a 2023 update on treatment options
• Australian and New Zealand consensus guideline for paediatric newly diagnosed immune thrombocytopaenia endorsed by Australian New Zealand Children’s Haematology and Oncology Group (2023)

The following guidance is available from international experts outside Australia; however, there may be information that is not relevant or applicable to the Australian context, and may not be up to date:

• Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group – developed by an International Working Group (IWG) of recognized experts; published in 2009
• Updated international consensus report on the investigation and management of primary immune thrombocytopenia – an update of the 2010 International Consensus Report; published in 2019 and involves Australian perspective

Individuals living with rare diseases may have complex medical issues and disabilities, which are not always visible. It is often useful to refer to their medical history as well as personal information such as a medical card, doctor’s letter, or if available, a rare disease passport, for relevant information.

In addition, individuals, their parents, families and carers often develop extensive expertise on their specific rare disease. It is important to recognise that they can contribute valuable knowledge about their rare condition. Rare diseases often impact individuals differently, so it’s important to consider a person’s lived experience.

ITP Australia and New Zealand’s ITP Wallet Card has information intended for healthcare professionals, including emergency department clinicians, nurses, and general practitioners who have someone with Immune Thrombocytopenia come to them seeking medical assistance.

Severe or life-threatening bleeding has been reported in some individuals with ITP.⁷ Consensus guidelines for the management of adult immune thrombocytopenia in Australia and New Zealand (2021) includes information about treatment of emergency bleeding in ITP.

Australian Organisation:

ITP Australia and New Zealand (ITPANZ)  
Website: https://itpaustralia.org.au

The mission at ITP Australia and New Zealand (ITPANZ) is to provide up-to-date information, support and advocacy for the ITP patient community. ITPANZ is also part of the International ITP Alliance.

Please note that RVA does not monitor or endorse each group/organisation’s operational governance and activities. When engaging with a group, please consider the information on the RARE Portal’s Finding Helpful Peer and Community Supports page.

Immune thrombocytopenia (ITP) varies between individuals, and each person’s experience is unique.

ITP Australia and New Zealand: Our ITP Stories has personal stories of people living with ITP.

If you would like to share your personal story with RVA, please visit the Rare Voices Australia: Share Your Story page. RVA will consider your story for publishing on our website and inclusion on the RARE Portal.

ITP Australia and New Zealand’s ITP Support Hub include the following support resources for people living with ITP:

• Newly Diagnosed Information Brochure
• ITP Wallet Card
• ITP Shared Decision-Making Toolkit (developed by ITP Support Association and UK ITP Forum via an expert working group) – helps you and your healthcare team work together to choose the treatment that feels right for you
• ITP Buzzy Kits – small vibrating device designed to help block sharp pain and provide distraction during medical procedures
• ITP Student Fact Sheet
• ITP Student Card
• ITP Conversation Cards
• ITP & Me: Teen Book

For information on available government and social services that provide support for individuals with a rare disease, please visit the National and State Services pages.

People living with a rare disease often face unique challenges such as diagnostic delays, misdiagnoses, limited treatment options, and limited access to rare disease specialists and support. These challenges may impact people’s emotional wellbeing and quality of life. Many find it helpful to seek mental health and wellbeing support to cope with ongoing stress and uncertainty. Connecting with people who have shared experiences through a support group may also be helpful. Information about relevant mental health and wellbeing support can be found at:

• Mental Health and Wellbeing Support for Australians Living with a Rare Disease
• The National and State Services pages underneath the ‘Mental Health’ sections listed

• ITP Australia and New Zealand: About ITP
• ITP Australia and New Zealand: ITP Videos
• Kids Health Info (The Royal Children’s Hospital Melbourne): Idiopathic thrombocytopenic purpura (ITP)
• Better Health Channel: Immune thrombocytopenic purpura (ITP)
• Genetic and Rare Diseases (GARD) Information Center: Autoimmune thrombocytopenic purpura
• ITP Podcast: Beyond the Count

Royal College of Pathologists of Australasia (RCPA): Immune thrombocytopenia

ITP Australia and New Zealand: Immune Thrombocytopenia Healthcare Professional Register

Orphanet: Immune thrombocytopenia

This page has been developed by Rare Voices Australia (RVA)’s RARE Portal team in consultation with ITP Australia and New Zealand.

If you are aware of any additional information that may benefit stakeholders with an interest in this page, or if you notice any broken links or inaccurate information, please let us know via the Contribute page.