Retrospective Analysis of Clinical and Pathological Characteristics of POEMS Syndrome in 11 Patients

  Objective  To summarize the clinical features and neuropathological characteristics of peripheral nerves in patients with POEMS syndrome.

  Methods  We retrospectively analyzed the clinical and neuropathological characteristics of 11 patients with POEMS syndrome who underwent electrophysiology and sural nerve biopsy in our department from January 2003 to December 2008.

  Results  Among these 11 patients, there were 8 men and 3 women, aged 40 to 64 years, with a disease course ranging from 8 months to 8 years. All patients suffered from sensorimotor polyneuropathy. Monoclonal immunoglobulins were detected in 10 patients, including IgAλ (n=6), IgGλ (n=2), and monoclonal λ light chain with unknown typing (n=2). Ten patients had organomegaly, 8 had endocrine disease, and 10 had skin changes. Biopsy of sural nerve presented loss of myelinated fibers. Five showed axonal degenation and six showed both axonal degeneration and demyelination. Changes in the endoneurial and epineurial microvessels, including hyperplasia of endothelial cells and widened basement membrane, were observed.

  Conclusion  Biopsy of the sural nerve in patients with POEMS syndrome shows both axonal degeneration and demyelination or axonal degeneration, as well as small ves-sel lesions without inflammation.