Facts about cTTP

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Interesting facts about cTTP you may not have known before

Learning some facts about cTTP can help you stay connected and discover more about it, in a simple way. Read on to find out more.

Fact #1: cTTP is a form of TTP but there is also a different form called iTTP1

TP (or thrombotic thrombocytopenic purpura) is a rare, life-threatening blood condition in which blood clots form throughout small blood vessels of the body.2 These blood clots can impair the normal flow of blood, leading to organ damage and other complications.2 A person can be born with this condition—due to an affected gene—in which case it is called congenital TTP (cTTP). However, they can also develop it later in life—due to their immune system response—in which case it is called immune-mediated or acquired TTP (iTTP).2 You can read more about the difference between cTTP and iTTP here.

A clump of red blood cells held together by strands that are pale red to form a clot. There are some singular red blood cells.

Fact #2: About 0.5 to 2 persons per million worldwide are thought to have cTTP1

cTTP is in fact, one of a select group of conditions sometimes referred to as “ultra-rare” as it is so uncommon.3

A crowd of adults, both men and women. Most are back and white but two at the front are in colour. The woman wears a blue top and long brown skirt, while the man wears a yellow t shirt and blue jeans.

Fact #3: There can, sometimes, be an average delay of almost 4 years before someone experiences their first symptoms and is then diagnosed with cTTP4

As cTTP is so rare, it may not be the first thing that comes to mind during medical or health consultations. Unfortunately, a delayed diagnosis – or a wrong diagnosis – might sometimes mean unnecessary tests and treatments for those affected. Click here to find out more about the symptoms of cTTP.

A man sits up in a hospital bed covered by a blue blanket with a blue top. A drip stand is beside the bed. A woman wearing a white doctor's coat is stood by the bed holding a clipboard.

Fact #4: Flare-ups of cTTP may be triggered by infections, some drugs, and pregnancy5

Consider speaking to your care team if you have any concerns or questions about your condition flaring up – such as if you are worried about whether it is safe to start a new medication.

A woman wearing a doctor's coat and brown trousers sits on a chair with a laptop on the table next to her. She is holding a pen and gesturing with her hand at a pregnant woman sat on the other side.

Fact #5: cTTP is also known as Upshaw-Schulman syndrome1

cTTP was discovered in the USA in the 1960s and 1970s, largely due to the efforts of two researchers called Upshaw and Schulman.6 This is why it has been named after them.

A man wearing a white doctor's coat sits on a stool looking through a microscope while another doctor stands nearby holding a clipboard.

Fact #6: cTTP occurs when a protein in the blood called ADAMTS13 is missing or at very low levels

An enzyme called ADAMTS13 helps control clotting of the blood.2 People with cTTP are born with a fault in the gene that makes ADAMTS13 in the body.1,2 The results in a deficiency of this enzyme.1 This ultimately means there is an increased risk of blood clotting.2 You can read more about cTTP here.

Concentric red circles in different shades represent a blood vessel and within it are red bloods cells. Some red blood cells are tied together, forming a clot.

Fact #7: The current treatments for cTTP are infusion therapies which aim to replace the missing blood protein ADAMTS137

These treatments need to be given in hospital and can take hours to administer.7 Scientists are searching for new treatments that can be given at home. You can ask your care team for any updates or information about cTTP treatments.

A woman sits up in a hospital bed. She's covered by a blue blanket. A drip stand with two drip bags containing blood is beside the bed. A woman wearing a white doctor's coat is stood by the bed.

Further reading

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Planning ahead when you travel

There are measures you can take to help make traveling with cTTP easier.

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Understanding your cTTP diagnosis

Discover what cTTP is and how it can affect you.

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The information provided on this website is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with any concerns or questions you may have regarding your medical condition or treatment.

References

  1. Sukumar S, et al. J Clin Med. 2021;10:536.
  2. National Heart, Lung, and Blood Institute. Available at: www.nhlbi.nih.gov/health/thrombotic-thrombocytopenic-purpura. Accessed August 2024.
  3. Sardella M, Belcher G. Therapeutic Advances in Drug Safety. 2018;9(11):631–638.
  4. Alwan F, et al. Blood. 2019;133(15):1644–1651.
  5. Tarasco E, et al. Blood. 2021;137(25):3563​–3575.
  6. The Royal College of Pathologists. Available at: https://www.rcpath.org/profession/publications/college-bulletin/october-2022/thrombotic-thrombocytopenic-purpura-past-present-and-future.html. Accessed August 2024.
  7. Sakai K, Matsumoto M. J Clin Med. 2023;12(10):3365.