Not just one disease but ‘a group’ of pathologieswith different symptoms and stories depending on the protein involved. Amyloidosis, which photographer Oliviero Toscani revealed he suffers from, it’s a rare diseasecharacterized by an abnormal accumulation of proteins that are deposited in various tissues of the body, damaging organs and causing dysfunctions that can even be fatal. The type and severity of symptoms depend on the vital organs affected.. To obtain a diagnosis, it is necessary to take a tissue sample (biopsy). In the many forms of amyloidosis, treatment depends on the type you are affected by.
The symptoms
Amyloid deposits, in fact, as explained for example in various focuses, including the dedicated chapter in the MSD manual, can be: systemic, that is, spread throughout the body, or localized in a single organ or tissue. The most common form of systemic amyloidosis in Western countries, reports the Mario Negri Institute, is that of immunoglobulin light chains, also called AL amyloidosis, with an incidence of about 10 new cases per million people per year. But in general, to date 37 amyloidogenic proteins have been identified and each of them is associated with a specific form of amyloidosis. There is great variability in the symptoms. Some people experience mild symptoms, while others develop serious, potentially life-threatening conditions.. Common symptoms of amyloidosis are fatigue and weight loss.. Other symptoms depend on the location of the deposits. The problems more serious occur when the heart is affected. Abnormal heart rhythms or heart failure may occur, causing shortness of breath, weakness, or fainting.
When nerves are affected, there may be tingling or numbness in the fingers and toes or dizziness when standing. When the kidneys are affected, there may be swelling (edema) on the feet and legs and sometimes on the abdomen. When the skin is affected, bruising is common, often around the eyes. In some cases, the tongue also swells. The prognosis depends on the type of amyloidosis and the organs affected. Involvement of the heart is the most dangerous and can have a poor prognosis.
Therapies
The therapeutic approach, as illustrated by the Italian Association against Leukemia, Lymphoma and Myeloma in an overview of amyloidosis, published on its portal, is similar to that used for multiple myeloma, although some drug combinations are slightly different. Eligibility for autologous stem cell transplantation is strongly influenced by cardiac damage. Recently, the advent of anti-CD38 monoclonal antibodies (such as daratumumab) has offered new therapeutic possibilities for this complex pathology, for which even diagnosis remains a challenge, both due to the low frequency of cases and the difficulty in recognizing the symptoms.
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