To describe clinical manifestations, diagnostic algorithms and treatment strategy of localized AL-amyloidosis (ALL ).
Material and methods
We recruited 30 patients with ALL
The most frequent types of ALL were laryngeal and tracheobronchial (n=12) and conjunctival (n=8) forms. The other sites of ALL included lungs (n=3), skin (n=3), urinary bladder (n=2), brain (n=1) and soft tissue (n=1). Amyloid deposits were tumor-like (n=17) or diffuse (n=13). The median time to diagnosis was 23 months (range 8-55 months). The absence of proteinuria and monoclonal gammopathy (high resolution immunoelectrophoresis and immunofixation of bone marrow) allowed to exclude systemic AL-amyloidosis. The relapse after surgical treatment was registered in 1⁄4 of patients.
The first-line treatment of ALL was excision of the amyloid deposits. However more efficient treatment strategies are needed (e.g. radiotherapy for localized plasma cell clone elimination) due to the high relapse rate.
Localized AL-amyloidosis, immunoelectrophoresis, immunofixation, radiotherapy.