Diagnosis and treatment of localized AL-amyloidosis

Aim

To describe clinical manifestations, diagnostic algorithms and treatment strategy of localized AL-amyloidosis (AL_L ).

Material and methods

We recruited 30 patients with AL_L

Results

The most frequent types of AL_L were laryngeal and tracheobronchial (n=12) and conjunctival (n=8) forms. The other sites of AL_L 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.

Conclusion

The first-line treatment of AL_L 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.

Key words

Localized AL-amyloidosis, immunoelectrophoresis, immunofixation, radiotherapy.