title: POEMS syndrome with opticneuritis as initial symptom: a case report.
abstract: Polyneuropathy, Organomegaly, Endocrinopathy, M-protein and Skin changes syndrome is a rare paraneoplastic syndrome from a plasma cell dyscrasia, which most commonly presents with peripheral neuropathy. We report a 30-year-old female with blurred vision and vision loss as reasons for her initial treatment; the patient was diagnosed with bilateral opticneuritis. Five months later, the patient started to have lower extremity numbness and fatigue. Initial laboratory tests did not reveal positive monoclonal immunoglobulin. Therefore, simple peripheral neuropathy was considered for diagnosis, and the patient was treated in the Department of Neurology in our hospital; however, the symptom was not alleviated. Seven months later, the patient showed symptoms of cough, dyspnea, along with rough skin, pigmentation and drooping of both feet. Chest CT revealed bronchiectasis and infection of lungs, destruction of thoracic bones and ribs. Further examination indicated that the patient had splenomegaly, hypothyroidism and monoclonal IgA-λ chain zone, which led to a clear diagnosis of Polyneuropathy, Organomegaly, Endocrinopathy, M-protein and Skin changes syndrome.
authors.affiliation: Doctor, Department of Endocrinology, The Huai-an First Affiliated Hospital of Nanjing Medical University, Jiangsu, China.
authors.forename: Yuanyuan
authors.initials: Y
authors.lastname: Liu
authors.affiliation: Doctor, Department of Endocrinology, The Huai-an First Affiliated Hospital of Nanjing Medical University, Jiangsu, China.
authors.forename: Weiping
authors.initials: W
authors.lastname: Lu
authors.affiliation: Doctor, Department of Endocrinology, The Huai-an First Affiliated Hospital of Nanjing Medical University, Jiangsu, China.
authors.forename: Hong
authors.initials: H
authors.lastname: Zhang
authors.affiliation: Doctor, Clinical Laboratory, Huai'an Hospital Affiliated of Xuzhou Medical College, Jiangsu, China lixiang_suda@163.com.
authors.forename: Xiang
authors.initials: X
authors.lastname: Li
mesh_terms: D000328:Adult; D018906:Antineoplastic Agents, Alkylating / Q000008:administration & dosage*; D003937:Diagnosis, Differential; D018450:Disease Progression; D004417:Dyspnea; D005260:Female; D006801:Humans; D008558:Melphalan / Q000008:administration & dosage*; D009902:Optic Neuritis / Q000175:diagnosis* / Q000209:etiology; D016878:POEMS Syndrome / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy; D010291:Paresis / Q000175:diagnosis* / Q000209:etiology; D036102:Peripheral Blood Stem Cell Transplantation; D016896:Treatment Outcome
chemical_list: D018906:Antineoplastic Agents, Alkylating; D008558:Melphalan
keywords: IgA λ monoclonal immunoglobulin; POEMS syndrome; optic neuritis; peripheral neuropathy; plasma cell disease