AL-Amyloidose – Aktuelle Diagnostik und Therapie
摘要
The AL amyloidosis is a disease in which the deposition of incorrectly folded monoclonal light chains can lead to a rapid progression of organ dysfunction. Most frequently affected are the heart and kidneys and somewhat less frequently liver, nervous system and gastrointestinal tract. Although the disease is relatively rare, substantial progress has been made in recent years with respect to the treatment of the underlying B cell disease. In contrast, results of studies with substances that should lead to a reduction in amyloid deposition are still pending. In addition, the early recognition of the disease coupled with a rapid diagnosis and initiation of treatment must be further improved.