Amyloidosis is considered a cancer

Amyloidosis - a rare disease with a diverse clinical picture

Such systemic amyloidosis however, occurs extremely rarely. Only one in 100,000 people develops new systemic amyloidosis every year. The patients are usually involved older than 65 years. Mild forms of amyloidosis are more common, though 800 new cases per year in Germany but still very rare.

The causes of amyloidosis

There are essentially three different forms of the disease. The Light chain (AL) amyloidosis arises as a result of diseases of the bone marrow. Here comes one Multiplication of the plasma cellsresponsible for the production of antibodies. In addition to the intact antibodies, structurally altered free light chains also always find their way into the blood. The body then deposits these in the organ structures. The replication of the plasma cells is often based on a tumor of the lymphatic tissue. AL amyloidosis is therefore particularly common in multiple myeloma or Waldenström's disease. Both diseases are malignant blood diseases.

The AA amyloidosis however, is through chronic inflammatory diseases evoked. The trigger here is the acute phase protein, which is increasingly formed in chronic infections such as tuberculosis or leprosy. Chronic inflammatory bowel diseases such as Crohn's disease or ulcerative colitis as well as rheumatic diseases can trigger AA amyloidosis. Changes in the genome and mutations can be a cause hereditary or familial amyloidosis. This occurs more often in combination with other inherited diseases such as familial Mediterranean fever.

Amyloidosis - the symptoms

The symptoms of the disease differ depending on the organ in which the protein threads are deposited. If this happens in the heart muscle, the organ stiffens and the blood supply to the body is reduced. Such Cardiomyopathy manifests itself through symptoms such as:

The protein threads can also accumulate in the kidney and lead to functional disorders there. The consequence is a nephrotic syndrome with protein loss about the kidney. This leads to water retention in the body and, if immunoglobulins, which are responsible for the defense system, are lost, also to increased infections. The amyloidosis of the gastrointestinal tract mainly makes itself felt Diarrhea, Bleeding or bowel obstruction with severe pain noticeable. The liver is enlarged and feels rock hard to the touch.

The brain and nerves can also be affected by the disease. This creates dysfunctions of all kinds, which affect the dementia resemble. The protein threads can also cause damage to the peripheral nerves, i.e. the nerves that lie outside the spinal cord and the brain. Typical symptoms here are sensitivity and movement disorders. The carpal tunnel syndrome, nodular changes in the skin, hair loss, red circles under the eyes or hoarseness are more likely to indicate one Soft tissue involvement down. In rare cases, the amyloidosis causes Disorders in the endocrine system which manifest themselves as thyroid disorders or underactive adrenal glands.

The diagnosis of amyloidosis

The symptoms of amyloidosis are often non-specific and can also occur with other diseases. Therefore, the diagnosis is often only made at an advanced stage of the disease. Amyloidosis is always suspected, however, when patients suffer from cardiomyopathy, nerve dysfunction or liver enlargement of unknown cause. Likewise, one should with patients with a multiple myeloma Always consider amyloidosis as a possible concomitant disease.

For a reliable diagnosis, the doctor must have a Tissue sample from an affected organ remove and examine in the laboratory. Here you can see the exact composition of the protein threads, which must be known for a successful treatment. In the case of generalized amyloidosis, the removal can also be carried out gently from the subcutaneous fatty tissue. If amyloidosis is confirmed by this examination method, a Bone marrow aspiration searched for causal diseases.

The extent of the thread deposits is recorded by a Scintigraphy. The patients receive radioactively marked substances that bind to the protein threads so that they can be seen in the imaging process. However, the diagnosis is often difficult, so that the diagnosis of amyloidosis can often only be made after death at autopsy.

The therapy of amyloidosis

The majority of amyloidoses are treatable today. The therapy depends on the form of the disease. So you can prevent the uncontrolled multiplication of plasma cells by a high-dose chemotherapy with following Stem cell transplantusually contain.

Patients with Mediterranean fever can develop amyloidosis by taking the substance Colchicine counteract. In addition, the organ damage is caused by various general measures and medications treated. People with heart disease are given ACE inhibitors and diuretics to lower blood pressure. If the kidneys are involved, a low-salt diet can be useful.

Amyloidosis - the prognosis

Whether and how well amyloidosis can be treated depends, on the one hand, on the form of the disease and, on the other hand, on the age of the patient. The presence of multiple myeloma or heart disease significantly worsens the prognosis, whereas mild forms of the disease are often only associated with a slightly reduced life expectancy.