POEMS syndrome and Castleman’s disease

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.