[Clinical features and prognosis of patients with peripheral neuropathy with systemic light chain amyloidosis]

The clinical data and prognosis of 6 patients with systemic light chain amyloidosis-related peripheral neuropathy admitted to Huashan Hospital Affiliated to Fudan University from June 2019 to January 2024 were analyzed retrospectively, and discussed.The patients were from 48 to 70 (58±9) years, including 4 males and 2 females. Among the initial symptoms, 4 cases had both lower limb sensory abnormalities, 1 case had both upper limb numbness, pain, weakness, 1 case had swallowing disorders, 5 cases had pain and autonomic symptoms, 6 cases had weight loss, 6 cases had heart involvement, mainfested as chest tightness, shortness of breath, and 3 patients had kidney involvement, manifested as asymptomatic proteinuria. Four cases had light chain type λ, 1 case had λ+κ and i case had κ. Five cases underwent lumbar puncture with protein cell separation and CSF protein was (1.00±0.21) g.Electrophysiological examination was performed in all 6 patients, suggesting multiple peripheral nerve damage involving motor and sensory nerves and mainly axonal involvement.Four cases (4/5) had positive intestinal biopsies; 1 (1/3) cases had positive nerve biopsy; 1 (1/1) case had positive skin biopsy and 1 (0/1) had negative fat biopsy; 5 (0/5) had bone marrow biopsy, with negative Congo red staining. All 6 patients underwent chemotherapy, with only 1 improving, 3 experiencing gradually worsening symptoms, and 2 dying. Multiple peripheral nerve axonal damage and autonomic nerve involvement are characteristics of systemic light chain amyloidosis peripheral neuropathy.