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 Correct connections are crucial
26.06.2017 | Charité - Universitätsmedizin Berlin

nachricht One gene closer to regenerative therapy for muscular disorders
01.06.2017 | Cincinnati Children's Hospital 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: Can we see monkeys from space? Emerging technologies to map biodiversity

An international team of scientists has proposed a new multi-disciplinary approach in which an array of new technologies will allow us to map biodiversity and the risks that wildlife is facing at the scale of whole landscapes. The findings are published in Nature Ecology and Evolution. This international research is led by the Kunming Institute of Zoology from China, University of East Anglia, University of Leicester and the Leibniz Institute for Zoo and Wildlife Research.

Using a combination of satellite and ground data, the team proposes that it is now possible to map biodiversity with an accuracy that has not been previously...

Im Focus: Climate satellite: Tracking methane with robust laser technology

Heatwaves in the Arctic, longer periods of vegetation in Europe, severe floods in West Africa – starting in 2021, scientists want to explore the emissions of the greenhouse gas methane with the German-French satellite MERLIN. This is made possible by a new robust laser system of the Fraunhofer Institute for Laser Technology ILT in Aachen, which achieves unprecedented measurement accuracy.

Methane is primarily the result of the decomposition of organic matter. The gas has a 25 times greater warming potential than carbon dioxide, but is not as...

Im Focus: How protons move through a fuel cell

Hydrogen is regarded as the energy source of the future: It is produced with solar power and can be used to generate heat and electricity in fuel cells. Empa researchers have now succeeded in decoding the movement of hydrogen ions in crystals – a key step towards more efficient energy conversion in the hydrogen industry of tomorrow.

As charge carriers, electrons and ions play the leading role in electrochemical energy storage devices and converters such as batteries and fuel cells. Proton...

Im Focus: A unique data centre for cosmological simulations

Scientists from the Excellence Cluster Universe at the Ludwig-Maximilians-Universität Munich have establised "Cosmowebportal", a unique data centre for cosmological simulations located at the Leibniz Supercomputing Centre (LRZ) of the Bavarian Academy of Sciences. The complete results of a series of large hydrodynamical cosmological simulations are available, with data volumes typically exceeding several hundred terabytes. Scientists worldwide can interactively explore these complex simulations via a web interface and directly access the results.

With current telescopes, scientists can observe our Universe’s galaxies and galaxy clusters and their distribution along an invisible cosmic web. From the...

Im Focus: Scientists develop molecular thermometer for contactless measurement using infrared light

Temperature measurements possible even on the smallest scale / Molecular ruby for use in material sciences, biology, and medicine

Chemists at Johannes Gutenberg University Mainz (JGU) in cooperation with researchers of the German Federal Institute for Materials Research and Testing (BAM)...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

Plants are networkers

19.06.2017 | Event News

Digital Survival Training for Executives

13.06.2017 | Event News

Global Learning Council Summit 2017

13.06.2017 | Event News

 
Latest News

Study shines light on brain cells that coordinate movement

26.06.2017 | Life Sciences

Smooth propagation of spin waves using gold

26.06.2017 | Physics and Astronomy

Switchable DNA mini-machines store information

26.06.2017 | Information Technology

VideoLinks
B2B-VideoLinks
More VideoLinks >>>