POEMS Syndrome and Castleman’s Disease

  • Angela Dispenzieri


POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. The dominant feature is typically peripheral neuropathy, and the hematologic manifestations can be subtle leading to delayed diagnosis. Although the mechanism of disease is not well understood, the disease is very treatable, and survival is typically excellent. Castleman’s disease (CD) can occur with and without POEMS syndrome. Patients with unicentric CD have an excellent prognosis with local therapies; outcomes are less good, and therapies are less well established for the patients with multicentric CD with the exception of interleukin-6 and interleukin-6 receptor antibodies, which are useful in a subset of patients.


POEMS syndrome Osteosclerotic myeloma Castleman’s disease Monoclonal gammopathy of undetermined significance Multiple myeloma Paraneoplastic syndrome Vascular endothelial growth factor 



AD and this work are support in part by the Robert A. Kyle Hematologic Malignancies Fund, the Predolin Foundation, the JABBS Foundation, and the Andrew & Lillian A. Posey Foundation.


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Copyright information

© Springer Science+Business Media New York 2017

Authors and Affiliations

  1. 1.Division of Hematology, Department of MedicineMayo ClinicRochesterUSA

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