Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

Survival without lasting damage

15.02.2016

Anyone who survives the rare blood disorder TTP is then left fighting the lingering neurological damage. So this does not remain the case, the Department of Hematology of Bern University Hospital is developing a faster treatment that is less likely to result in a relapse.

The rare and life-threatening blood disorder thrombotic thrombocytopenic purpura (TTP) mainly affects young, otherwise healthy people aged between 20 and 50. Only 2-3 people out of every million will fall ill with TTP, which forms blood clots in the small blood vessels.


Schematic presentation of platelets (purple) which stick to the over-long Von Willebrand factor (brown).

Massachusetts Medical Society


Blood smear of patient with TTP under the microscope.

Department of Hematology, Inselspital, Bern University Hospital

The decreased blood flow damages the heart, brain and kidneys in particular and can lead to strokes and heart attacks. The disorder mainly affects women and will lead to death in over 90 percent of cases if it is not treated within a few days.

Autoimmune disorder with lasting consequences

TTP is an autoimmune disorder with antibodies against the ADAMTS13 protein enzyme. Through the acute lack of ADAMTS13, the von Willebrand factor is no longer regulated in terms of size and blood platelets spontaneously adhere to it. For this reason, the standard treatment at the moment consists of daily plasma exchange (removal of antibodies, supply of ADAMTS13) and immunosuppressive drugs.

Despite the treatment, 10-20 percent of patients die during the acute episode. More than half of patents are left with neurological damage as a result of the decreased blood flow, such as impaired concentration, attention deficit and visual problems, numbness in an arm or leg and paralysis of these. With almost half, the disorder flares up again within 1-2 years.

Rapid control of the disorder with lower rate of relapses

In a multicentre clinical study (CH, AU, IT, BE, USA) with the involvement of the Department of Hematology of the Bern University Hospital, it has now been possible to treat TTP with an anti-von Willebrand factor nanobody, which prevents the blood platelets from adhering. As a result, TTP can be forced back within a few days and the organs are protected from further decreased blood flow – which prevents at least some of the remaining damage.

75 patients were involved in the study. Together with the standard treatment, 36 patients received the new active ingredient and the 39 patients of the control group received a placebo. Bern-based haematologist Johanna Kremer Hovinga analysed all the blood samples and found that in 95 percent of patients, who received the new active ingredient, the acute phase of TTP had ended after scarcely 4 days, making this almost 40 percent faster than with the traditional treatment.

The side-effects were generally comparable, but light bleeding occurred more frequently with the new active ingredient (54% of patients in comparison with 38% with the placebo). As long as the medication was given, no patient had a relapse, although the autoimmune reaction continued to be active in at least 20 percent of patients. Another advantage: Because the new active ingredient can be injected subcutaneously, it was possible to treat patients on an outpatient basis after just a few days.

Long-term special subject at the Bern University Hospital and Bern University

The Department of Hematology at the Bern University Hospital and the University of Bern have been researching TTP and ADAMTS13 since the mid-1990s and have published extensive work in this. This study in the New England Journal of Medicine is the first major randomised patient study in the field of the rare disorder of TTP and shows a promising new treatment approach based on expanded knowledge of TTP.

Contact: Prof. Dr.med. Johanna Kremer Hovinga Strebel, senior consultant, Department of Hematology and Central Hematology Laboratory, Bern University Hospital, +41 31 632 02 65.

Weitere Informationen:

http://www.insel.ch/en/
http://www.nejm.org/doi/full/10.1056/NEJMoa1505533

Monika Kugemann | Universitätsspital Bern

More articles from Health and Medicine:

nachricht GLUT5 fluorescent probe fingerprints cancer cells
20.04.2018 | Michigan Technological University

nachricht Scientists re-create brain neurons to study obesity and personalize treatment
20.04.2018 | Cedars-Sinai Medical Center

All articles from Health and Medicine >>>

The most recent press releases about innovation >>>

Die letzten 5 Focus-News des innovations-reports im Überblick:

Im Focus: Spider silk key to new bone-fixing composite

University of Connecticut researchers have created a biodegradable composite made of silk fibers that can be used to repair broken load-bearing bones without the complications sometimes presented by other materials.

Repairing major load-bearing bones such as those in the leg can be a long and uncomfortable process.

Im Focus: Writing and deleting magnets with lasers

Study published in the journal ACS Applied Materials & Interfaces is the outcome of an international effort that included teams from Dresden and Berlin in Germany, and the US.

Scientists at the Helmholtz-Zentrum Dresden-Rossendorf (HZDR) together with colleagues from the Helmholtz-Zentrum Berlin (HZB) and the University of Virginia...

Im Focus: Gamma-ray flashes from plasma filaments

Novel highly efficient and brilliant gamma-ray source: Based on model calculations, physicists of the Max PIanck Institute for Nuclear Physics in Heidelberg propose a novel method for an efficient high-brilliance gamma-ray source. A giant collimated gamma-ray pulse is generated from the interaction of a dense ultra-relativistic electron beam with a thin solid conductor. Energetic gamma-rays are copiously produced as the electron beam splits into filaments while propagating across the conductor. The resulting gamma-ray energy and flux enable novel experiments in nuclear and fundamental physics.

The typical wavelength of light interacting with an object of the microcosm scales with the size of this object. For atoms, this ranges from visible light to...

Im Focus: Basel researchers succeed in cultivating cartilage from stem cells

Stable joint cartilage can be produced from adult stem cells originating from bone marrow. This is made possible by inducing specific molecular processes occurring during embryonic cartilage formation, as researchers from the University and University Hospital of Basel report in the scientific journal PNAS.

Certain mesenchymal stem/stromal cells from the bone marrow of adults are considered extremely promising for skeletal tissue regeneration. These adult stem...

Im Focus: Like a wedge in a hinge

Researchers lay groundwork to tailor drugs for new targets in cancer therapy

In the fight against cancer, scientists are developing new drugs to hit tumor cells at so far unused weak points. Such a “sore spot” is the protein complex...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

VideoLinks
Industry & Economy
Event News

Invitation to the upcoming "Current Topics in Bioinformatics: Big Data in Genomics and Medicine"

13.04.2018 | Event News

Unique scope of UV LED technologies and applications presented in Berlin: ICULTA-2018

12.04.2018 | Event News

IWOLIA: A conference bringing together German Industrie 4.0 and French Industrie du Futur

09.04.2018 | Event News

 
Latest News

Magnetic nano-imaging on a table top

20.04.2018 | Physics and Astronomy

Start of work for the world's largest electric truck

20.04.2018 | Interdisciplinary Research

Atoms may hum a tune from grand cosmic symphony

20.04.2018 | Physics and Astronomy

VideoLinks
Science & Research
Overview of more VideoLinks >>>