The Interdisciplinary Amyloidosis Center of the Medical University of Vienna / General Hospital of the City of Vienna is an interdisciplinary group of experts working closely together to ensure efficient diagnosis and optimal treatment.
Efficient diagnostics and optimal therapy through close cooperation. The intensive cooperation gives us the opportunity to provide our patients with individual and intensive care, thereby thus improving quality of life and increasing life expectancy.
The interdisciplinary collaboration between the Departments of Haematology, Cardiology, Nephrology, Pathology, Nuclear Medicine and Radiology has been in place since October 2012.
There are also joint projects with other universities and other amyloidosis centers in Europe and the USA.
In December 2019, the amyloidosis working group at the Medical University of Vienna was recognized as an official associated amyloidosis center (ERN: EuroBloodNet) by the Ministry of Social Affairs, Health, Care and Consumer Protection.
Amyloidosis in general:
Generally speaking, amyloidosis is a disease caused by an incorrectly formed protein. Due to the incorrect structure, the protein in question is deposited in the tissues. Cardiac deposits are the most threatening because they severely disrupt heart function (and often lead to cardiac arrhythmia and death). Protein deposits in the kidneys lead to an increased loss of protein and albumin via the kidneys. Protein deposits in the gastrointestinal tract and liver lead to frequent diarrhea, weight loss, GI bleeding and abdominal discomfort.
The diagnosis of amyloidosis can - with only a few exceptions - only be confirmed by means of a biopsy and a corresponding histological examination. A total of 36 forms of amyloidosis are currently known and the treatments vary greatly depending on which amyloidosis is involved. Therefore, not only must it be certain that amyloidosis is present, but it must also be clearly typed which protein is deposited in the organs in order to ensure the correct therapy.
The 3 most common forms of amyloidosis are:
Light chain/AL amyloidosis:
This develops as part of a plasma cell disease or a lymphatic disease that is associated with the formation of a paraprotein (MGUS, SMM, MM, follicular lymphomas, MALT lymphomas, Waldenström's disease). This is an essential prerequisite for the development of AL/light-chain amyloidosis.
Treatment is carried out by means of combined immuno-chemotherapy. If possible, a combination with high-dose chemotherapy with stem cell support should be carried out (in cooperation with Hanusch Hospital). In special cases, an organ transplant may also be necessary. We cooperate closely with our transplant center for this purpose.
Transthyretin amyloidosis/ATTR:
A distinction is made between 2 forms of transthyretin/ATTR amyloidosis: The genetically determined congenital form (ATTRv) and the form acquired in the course of life (ATTRwt). In both cases, transthyretin chains are deposited. The deposition of transthyretin in the heart does not require a biopsy, but can be detected by means of a nuclear medical examination (DPD scan). Genetic testing is necessary to rule out or detect congenital amyloidosis. This examination can be carried out on peripheral blood.
Serum amyloid A amyloidosis/AA:
This form of amyloidosis is caused by deposits of incorrectly folded serum amyloid A chains. AA amyloidosis develops in the context of chronic inflammatory diseases that persist for years and are not adequately treated or are very difficult to treat. Due to the chronic inflammation, the proteins from the group of acute-phase proteins are also elevated for years and are thus deposited in the tissue (mainly in the kidneys). Only rarely does AA also lead to cardiac involvement. Therapy is based on efficient treatment of the underlying chronic inflammatory disease.
Depending on which form of amyloidosis is present, the main care is provided by different departments at MedUni Vienna:
- AL amyloidosis patients at the Department of Hematology
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- ATTR patients at the Department of Cardiology
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- AA Amyloidosis at the Department of Nephrology, Rheumatology, Gastro/Enterology
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Control Center 6i, Level 6
T: +43 (0)1 40400-44640
Secretariat:
Renate Schoder, renate.schoder@meduniwien.ac.at
Jennifer Scherzer, jennifer.scherzer@meduniwien.ac.at
amyloidose@meduniwien.ac.at
Medical management:
Univ. Lektorin Priv-Doz.in Dr.in Hermine Agis
Assoc.-Prof.in Priv.-Doz.in Dr.in Maria Krauth