Open AccessAtypical polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes syndrome without polyneuropathy
Author(s) -
Qiang Li,
Fang Xu,
Jingfeng Duan,
Yufeng Tang
Publication year - 2020
Publication title -
medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.59
H-Index - 148
eISSN - 1536-5964
pISSN - 0025-7974
DOI - 10.1097/md.0000000000020812
Subject(s) - organomegaly , poems syndrome , medicine , polyneuropathy , plasma cell dyscrasia , pathology , dermatology , surgery , gastroenterology , immunology , antibody , immunoglobulin light chain
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.