Abstract
POEMS syndrome is a rare, chronic, disabling paraneoplastic disorder characterized by peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasma cells disorder and skin changes. Diagnosis relies on the fulfillment of a set of clinical criteria of which polyneuropathy and a monoclonal plasma cell dyscrasia are early and essential features. Treatment may be either local or systemic and is aimed at the monoclonal plasma cell disorder. Our knowledge of the pathogenesis underlying the POEMS syndrome has advanced greatly over the past years, favoring an important progression in the recognition and management of this disorder. Here, we discuss the recent literature that has advanced our knowledge of the pathogenesis and clinical management of the polyneuropathy in POEMS syndrome.
Highlights
POEMS syndrome is a rare multisystemic paraneoplastic disorder associated with osteosclerotic myeloma and increased serum and plasma levels of vascular endothelial growth factor (VEGF) [1,2,3,4,5,6,7]
The diagnosis of POEMS syndrome is confirmed when both the polyneuropathy and monoclonal gammopathy are present in association with one of the other three major criteria (Castleman disease, sclerotic bone lesions and increased levels of VEGF), and one of the six minor criteria
We propose that the mechanism underlying the peripheral neuropathy in POEMS syndrome is due to endothelial injury, indirectly or directly caused by an abnormal activation of endothelial cells by VEGF, which is overexpressed in the nerves of patients with POEMS syndrome, thereby inducing microvascular changes and impaired vascular permeability [7, 17, 20, 89]
Summary
POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome is a rare multisystemic paraneoplastic disorder associated with osteosclerotic myeloma and increased serum and plasma levels of vascular endothelial growth factor (VEGF) [1,2,3,4,5,6,7]. The diagnosis of POEMS syndrome is confirmed when both the polyneuropathy and monoclonal gammopathy are present in association with one of the other three major criteria (Castleman disease, sclerotic bone lesions and increased levels of VEGF), and one of the six minor criteria (organomegaly, endocrinopathy, extravascular volume overload, skin changes, papilloedema, thrombocytosis/polycythemia). We propose that the mechanism underlying the peripheral neuropathy in POEMS syndrome is due to endothelial injury, indirectly or directly caused by an abnormal activation of endothelial cells by VEGF, which is overexpressed in the nerves of patients with POEMS syndrome, thereby inducing microvascular changes and impaired vascular permeability [7, 17, 20, 89] These alterations lead to a secondary ischemic microangiopathy and chronic axonal damage of nerve fibers through alteration of the blood–nerve barrier [20]. Pain may be a dominant feature in patients with POEMS and should be treated pharmacologically with drugs such as gabapentin and tricyclic antidepressants
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