Treatment of AL amyloidosis
AL-type amyloidosis is caused by the deposition of subunits of defense proteins, the so-called immunoglobulin light chains. These light chains are formed by pathologically altered immune cells in the bone marrow or the lymph glands (plasma cells/lymph cells). AL amyloidosis is closely related to the development of tumorous diseases of the bone marrow and lymph glands, such as multiple myeloma and non-Hodgkin’s lymphoma. As with these diseases, treatment therefore also consists of systemic immuno-chemotherapy. This is intended to prevent the further production of light chains and the further formation of AL amyloid. The intensity of this chemotherapy depends on the patient’s resilience and general health. The organs most frequently affected are the heart, kidneys, nerves and intestines.
Treatment of hereditary and genetic amyloidosis
Hereditary amyloidosis is rare. However, there is an accumulation of patients in different regions of the world. Switzerland is not specifically affected. The cause of the disease is usually the faulty production of a protein that is formed in the liver. This transport protein is called transthyretin and the disease transthyretin (ATTR) amyloidosis. The main areas affected by the deposits are the nerves, the heart, the intestines and the eyes. Since transthyretin is produced in the liver and treatment is intended to prevent further production of this protein, liver transplantation is an important treatment option. Transthyretin amyloidosis can also occur as a new genetic change without being inherited from a parent. In addition to ATTR amyloidosis, there are other genetic amyloidoses. However, these are even rarer.
Amyloidosis network
The amyloidosis network at the University Hospital Zurich was founded in 2013 by our interdisciplinary team of specialists from the Departments of Gastroenterology, Hematology, Cardiology, Nephrology, Neurology, Neuropathology, Pathology and Rheumatology, the Institute of Medical Genetics at the University of Zurich and the Cantonal Hospital of Graubünden. Our aim is to extend and improve the lifespan and quality of life of affected people. Patients are cared for in close cooperation with the referring doctors.
