Atypical polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes syndrome without polyneuropathy

Abstract

Introduction: Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome is a rare paraneoplastic syndrome that occurs secondary to an underlying plasma cell disorder. The diagnosis of POEMS syndrome is 2 of the mandatory major criteria (polyneuropathy and monoclonal plasma cell disorder), 1 of the other major criteria (Castleman's disease, sclerotic bone lesions, or vascular endothelial growth factor elevation), and at least 1 of the minor criteria (organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema, thrombocytosis, or polycythemia). However, some cases do not fully meet the diagnostic criteria, such cases are referred to as incomplete or atypical POEMS syndrome. Patient concerns: A 58-year-old Chinese female was admitted to our department of neurology with weakness of both arms and legs. In addition,it's found that she had skin manifestations, lymphadenopathies, pedal edema, immunoglobin - A-λ restricted paraproteinemia, and elevated vascular endothelial growth factor and other features , b ut without polyneuropathy. Diagnoses: we made a diagnosis of atypical POEMS syndrome without polyneuropathy. Interventions: Two drug regimens were recommended: VAD (Vincristine, Adriamycin, Dexamethasone) and bortezomib. Finally, the VAD strategy was performed. Outcomes: The patient's limb strength and pain improved and enzyme parameters decreased gradually after 4 weeks. However, the treatment was still not perfect. Conclusion: We reported a rare case of POEMS syndrome without polyneuropathy. We hope similar cases will be reported in the future.

A case report on POEMS syndrome with high parathyroid hormone and repeated surgery, a rare presentation of a rare disease

“…However, not all the features are required to make the diagnosis [ 7 ]. A diagnosis of POEMS syndrome is confirmed when patients demonstrate both mandatory major criteria, one of the three other major criteria, and one of the six minor criteria [ 8 ].…”