POEMS syndrome presenting with abdominal distension, lower limb edema and shortness of breath: A case report and literature review

• Published in: Zhong nan da xue xue bao. Journal of Central South University. Yi xue ban Vol. 44; no. 6; p. 706
• Main Authors: Zhang, Jiayi, Ouyang, Zeying, Li, Rong, Leng, Aiming, Liu, Ting
• Format: Journal Article
• Language: English
• Published: China 28.06.2019
• Subjects: China; Dyspnea; Edema; Female; Humans; Lower Extremity; Middle Aged; POEMS Syndrome; Quality of Life; Vascular Endothelial Growth Factor A; China
• ISSN: 1672-7347
• DOI: 10.11817/j.issn.1672-7347.2019.06.015

POEMS syndrome is a rare paraneoplastic disorder. A 60-year-old female patient was admitted to the Department of Gastroenterology, Xiangya Hospital of Central South University (Changsha, China), complaining of abdominal distension, severe edema of both lower limbs and shortness of breath for more than 1 year. After intensive and careful medical investigations, the patient manifested with polyneuropathy, M-proteinemia, splenomegaly, lymphadenopathy, hypothyroidism, extravascular volume overload, sclerotic bone lesions, elevated VEGF and pulmonary hypertension. According to the latest diagnostic criteria of POEMS syndrome, this patient met two mandatory major criteria, two other major criteria and three minor criteria, the diagnosis was clear after ruling out differential diagnosis. The patient was treated with dexamethasone and lenalidomide, which relieved her clinical symptoms. The pathogenesis of POEMS syndrome is not fully understood; however, increased levels of vascular endothelial growth factor may contribute to most of the clinical manifestations. This patient had been in physical discomfort for more than 14 months, which seriously affected her quality of life. Clinically, the awareness of early diagnosis and treatment of POEMS syndrome should be improved.