Objective: This study aimed to better understand the clinical, electrophysiological, pathological features and prognosis of peripheral nerve involvements in primary
immunoglobulin light-chain (
AL) amyloidosis. Methods: We retrospectively reviewed the clinical data of eight
AL amyloidosis patients with
peripheral neuropathy as the initial presentation including clinical features, histopathological findings and treatment. Results: There were seven males and one female aged from 52 to 66 years. Initial symptoms included symmetrical lower extremity
numbness, lower extremity
pain and
carpal tunnel syndrome. Seven patients suffered from severe
pain and required
pain management. Six patients had predominant autonomic dysfunction. Six patients had cardiac involvement, and one patient had renal involvement. Monoclonal
proteins were found in all patients, with
IgA λ in one,
IgG λ in two, λ alone in three, κ alone in one and
IgM κ in one. Sural nerve biopsies were performed in 7 cases, all of which showed
amyloid deposition in the endoneurium (in the perivascular region in some cases), in addition to moderate to severe myelinated fiber loss with axonal degeneration. Six patients were treated with combined
chemotherapy. In three patients who began
chemotherapy earlier (6-10 months after onset), two achieved a hematological complete response, and one achieved a partial response. three patients who had delayed
chemotherapy (36 months after onset) died between 5 and 12 months after diagnosis. Conclusion: Early recognition of
AL amyloidosis with
peripheral neuropathy as the initial symptom is very important. Nerve biopsy can help to make the diagnosis. Early diagnosis and
chemotherapy are critical to achieve better outcomes.