Poems syndrome and paraproteinemic syndromes: Management and follow-up

Angela Dispenzieri, Suzanne R. Hayman

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Introduction POEMS syndrome is an atypical plasma cell disorders, composed of a diverse group of diseases that often share little other than an underlying plasma cell clone. The pathophysiologic relationship between the plasma cell dyscrasia is often cryptic in many of these disorders. However, understanding that there may be relationships between bone marrow and distant organ systems is important to arrive at a diagnosis and management plan. A convenient means of characterizing these disorders is by their dominant clinical feature: neuropathy, dermopathy and nephropathy (Figure 17.1). Some atypical plasma cell disorders will have phenotypes that cross many systems, as will be specified in the following pages. The most common atypical plasma cell disorder, light chain (AL) amyloidosis, may affect almost any organ system but will be discussed in a separate chapter. Atypical PCD with peripheral neuropathy as dominant phenotype Chemotherapy-induced peripheral neuropathy, Waldenstrom’s macroglobulinemia, multiple myeloma, and light chain amyloid are important etiologies for plasma cell disorder (PCD)-associated neuropathy, but these are beyond the scope of this review. In this section the focus will be upon POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) syndrome and MGUS (monoclonal gammopathy of undetermined significance) associated peripheral neuropathy.

Original languageEnglish (US)
Title of host publicationMyeloma: Pathology, Diagnosis, and Treatment
PublisherCambridge University Press
Pages225-244
Number of pages20
Volume9781107010574
ISBN (Print)9780511862465, 9781107010574
DOIs
StatePublished - Jan 1 2011

ASJC Scopus subject areas

  • Medicine(all)

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    Dispenzieri, A., & Hayman, S. R. (2011). Poems syndrome and paraproteinemic syndromes: Management and follow-up. In Myeloma: Pathology, Diagnosis, and Treatment (Vol. 9781107010574, pp. 225-244). Cambridge University Press. https://doi.org/10.1017/CBO9780511862465.017