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Health condition

TTP guide

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening emergency in which tiny clots form throughout the circulation because the ADAMTS13 enzyme is missing; immediate plasma exchange and modern targeted therapy have transformed survival.

Why trust this guide

  • 01

    Editorial team

    Written by our editorial team from the UK sources listed on each page.

  • 02

    Sourced from guidance

    Checked against current NHS and UK specialist society guidance at the time of review.

  • 03

    Current for 2026

    Reviewed on 2026-09-27 with the next review scheduled for 2027-09-27.

Key facts

The essentials at a glance.

The symptoms to recognise, when to seek help, which specialist to see, and what treatment usually involves.

  • Caused by severe ADAMTS13 deficiency, usually from an autoantibody

  • Suspect with anaemia, very low platelets and red cell fragments on the blood film

  • Treatment - plasma exchange, steroids, rituximab and caplacizumab - starts on suspicion

  • Survivors need long-term ADAMTS13 monitoring because relapse is common

Understanding the condition

What it is and what happens next.

What causes it, how it is diagnosed, and the treatment routes available in the UK.

What it is and how it feels

TTP is one of the few haematological diagnoses where hours matter. Without ADAMTS13, ultra-large strands of von Willebrand factor shred platelets and red cells into microclots that starve the brain, heart and kidneys. Presentations vary - confusion, fluctuating neurology, chest pain, dark urine, bruising - and the classic full pentad is rare, so the trigger for action is the blood picture, not the textbook syndrome.

Diagnosis and treatment routes

Daily plasma exchange replaces the missing enzyme and removes antibody; steroids and rituximab switch off production; caplacizumab, an anti-VWF agent, protects organs while that happens. With this package most patients survive what was once a routinely fatal disease. Follow-up matters just as much: ADAMTS13 activity is tracked in remission, and falling levels can prompt pre-emptive rituximab to prevent relapse. In many parts of the UK, routine NHS referrals for this kind of problem can take months, whereas an initial appointment with a private consultant is typically available within around two weeks.

Seeing a specialist

Who to see, and how quickly you can be seen.

The specialist for this condition is usually a consultant haematologist. Your GP can refer you on the NHS, or you can arrange a private consultation directly.

A quiet reminder

This guide is for information, not medical advice.

Your GP knows your history and can tell you which parts apply to you. If in doubt, see them - and seek urgent care for any of the emergency symptoms described above.

Frequently asked

Common questions about TTP guide.

Quick, plain-English answers to what people ask us most.

  • What is TTP (thrombotic thrombocytopenic purpura)?

    TTP is a rare, serious blood disorder in which small blood clots form throughout the body, using up platelets and damaging red blood cells, and reducing blood flow to organs.

  • What causes TTP?

    Most cases are caused by a severe deficiency of an enzyme called ADAMTS13, either due to an autoimmune antibody blocking it (acquired TTP) or, rarely, an inherited gene mutation (congenital TTP).

  • What are the symptoms of TTP?

    Symptoms can include unusual bruising or bleeding, fatigue, confusion or neurological symptoms, fever, and reduced urine output, developing over days, and can progress rapidly without treatment.

  • Is TTP dangerous?

    Yes, TTP is a medical emergency; without prompt treatment it can be fatal due to clots affecting the brain, heart, or kidneys, but survival rates are high with timely treatment.

  • Is TTP hereditary?

    The acquired form (most common) is not inherited and is caused by an autoimmune process, while congenital TTP is a rare inherited condition present from birth due to a faulty ADAMTS13 gene.

  • Can TTP be cured?

    Acquired TTP can go into remission with treatment, though relapse can occur and some people need long-term monitoring; congenital TTP requires ongoing management rather than a cure, often with regular plasma infusions.

  • How is TTP treated?

    Urgent treatment with plasma exchange (removing and replacing plasma) is the mainstay, often combined with immune-suppressing medication and a newer targeted drug called caplacizumab in acquired cases.

  • How long does TTP treatment take?

    Plasma exchange is typically continued daily until blood counts normalise, often one to two weeks, with additional immune therapy continuing for longer and regular monitoring afterward for relapse.

  • TTP vs haemolytic uraemic syndrome (HUS) - what's the difference?

    TTP is primarily caused by ADAMTS13 deficiency and often involves more neurological symptoms, while HUS is more commonly triggered by certain bacterial infections and predominantly affects the kidneys; specific blood tests help distinguish them, which is important because treatment differs.

  • When should I seek urgent care for suspected TTP?

    Suspected TTP is a medical emergency requiring immediate hospital assessment via A&E, as prompt treatment significantly improves survival.