August 31, 2015: Unresponsive CIDP: Is it POEMS?
August 31, 2015
Unresponsive CIDP: Is it POEMS?
POEMS is a plasma cell malignancy-associated paraneoplastic syndrome. It stands for polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. POEMS neuropathy can present similarly to CIDP, but with resistance to immunomodulatory treatment. SPEP shows a monoclonal protein, predominantly Lambda. Further evaluation should include a bone scan to evaluate for sclerotic lesions, a bone marrow biopsy to look for plasmacytoma, and VEGF levels which indicate disease activity. Treatment depends on the degree of bone marrow involvement, and includes radiotherapy, chemotherapy and stem cell transplantation.
- Mauermann, ML. Paraproteinemic Neuropathies. CONTINUUM 2014; 20: 1307-1322.
- Kuwabara S, Misawa S, Kanai K, et al. Neurologic improvement after peripheral blood stem cell transplantation in POEMS syndrome. Neurology 2008; 71: 1691–1695.
Submitted by Ahmed Yassin, MD, PGY4 Academic chief resident, Department of Neurology, University of Texas Medical Branch, Galveston, TX.
Disclosures: Dr. Yassin reports no disclosures.