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17 - POEMS syndrome and paraproteinemic syndromes: management and follow-up

from Section 3 - Myeloma: clinical entities

Published online by Cambridge University Press:  18 December 2013

By
Angela Dispenzieri  and
Suzanne R. Hayman
Edited by
Stephen A. Schey ,
Kwee L. Yong ,
Robert Marcus  and
Kenneth C. Anderson
Stephen A. Schey
Affiliation:
Department of Haematology, King’s College Hospital, London
Kwee L. Yong
Affiliation:
Department of Haematology, University College Hospital, London
Robert Marcus
Affiliation:
Department of Haematology, King’s College Hospital, London
Kenneth C. Anderson
Affiliation:
Dana-Farber Cancer Institute, Boston
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Summary

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.

Type
Chapter
Information
Myeloma
Pathology, Diagnosis, and Treatment
 , pp. 225 - 244
Publisher: Cambridge University Press
Print publication year: 2013

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