Li JR, Feng LY, Li JW, Liao Y, Liu FQ. Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes syndrome with dilated cardiomyopathy: A case report. World J Clin Cases 2024; 12(3): 601-606 [PMID: 38322456 DOI: 10.12998/wjcc.v12.i3.601]
Corresponding Author of This Article
Fei-Qi Liu, MD, Chief Physician, Department of General Medicine, Xiangtan Central Hospital, No. 120 Heping Road, Xiangtan 411100, Hunan Province, China. lfq720104@163.com
Research Domain of This Article
Hematology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Clin Cases. Jan 26, 2024; 12(3): 601-606 Published online Jan 26, 2024. doi: 10.12998/wjcc.v12.i3.601
Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes syndrome with dilated cardiomyopathy: A case report
Jia-Rong Li, Lei-Yu Feng, Jian-Wei Li, Yu Liao, Fei-Qi Liu
Jia-Rong Li, Lei-Yu Feng, Jian-Wei Li, Yu Liao, Fei-Qi Liu, Department of General Medicine, Xiangtan Central Hospital, Xiangtan 411100, Hunan Province, China
Author contributions: Li JR was responsible for data collection, manuscript writing and editing; Feng LY participated in data collection and editing; Li JW and Liao Y participated in editing; Liu FQ was responsible for conceptualization and supervision; all authors have read and approved the final manuscript.
Informed consent statement: All study participants, or their legal guardian, provided informed written consent prior to study enrollment.
Conflict-of-interest statement: The authors declare that they have no conflict of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Fei-Qi Liu, MD, Chief Physician, Department of General Medicine, Xiangtan Central Hospital, No. 120 Heping Road, Xiangtan 411100, Hunan Province, China. lfq720104@163.com
Received: October 11, 2023 Peer-review started: October 11, 2023 First decision: December 5, 2023 Revised: December 16, 2023 Accepted: January 4, 2024 Article in press: January 4, 2024 Published online: January 26, 2024 Processing time: 98 Days and 21.1 Hours
Abstract
BACKGROUND
Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a rare paraneoplastic syndrome that encompass multiple systems. The most common clinical symptoms of POEMS syndrome are progressive sensorimotor polyneuropathy, organ enlargement, endocrine disorders, darkening skin, a monoclonal plasma cell proliferative disorder, and lymph node hyperplasia. The organomegaly consists of hepatosplenomegaly and/or lymphadenopathy; cases of cardiomyopathy are rare. Diagnoses are often delayed because of the atypical nature of the syndrome, exposing patients to possibly severe disability. Therefore, identifying atypical symptoms can improve the prognosis and quality of life among POEMS syndrome patients.
CASE SUMMARY
Herein, we report the case of a 59-year-old woman with POEMS syndrome that involved dilated cardiomyopathy. The patient presented to the hospital with complaints of shortness of breath and discomfort in the chest. The patient reported previous experiences of limb numbness. During hospitalization, the brain natriuretic peptide levels were 3504.0 pg/mL. Color doppler echocardiography showed an enlarged left side of the heart, along with ventricular wall hypokinesis and compromised functioning of the same side of the heart. Abdominal color ultrasonography revealed that the patient’s spleen was enlarged. Observations from cardiac magnetic resonance imaging showed that the left side of the heart was enlarged. Slight myocardical fibrosis was also observed. Electromyography was described as a symmetric sensorimotor demyelinating polyneuropathy. Further immunoelectrophoresis of the serum showed the presence of a monoclonal IGA λ M protein. The vascular endothelial growth factor levels were 622.56 pg/mL. Flow cytometric and immunohistochemical staining of the bone marrow detected no monoclonal plasma cells. Finally, the patient was diagnosed with POEMS syndrome associated with dilated cardiomyopathy. The chest-related discomfort and the shortness of breath resolved after the administration of lenalidomide and dexamethasone.
CONCLUSION
When patients with cardiomyopathy have systemic manifestations such as numb limbs and darkening skin, the POEMS syndrome is the most possible diagnosis.
Core Tip: Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a rare paraneoplastic syndrome that encompass multiple systems. Diagnoses are often delayed because the unusual and atypical nature of the syndrome, exposing patients to possibly severe disability. Our report presents the first case of a 59-year-old Chinese female with newly diagnosed dilated cardiomyopathy and POEMS syndrome. Although POEMS syndrome with dilated cardiomyopathy is rare, physicians should consider it when patients with cardiomyopathy have systemic manifestations.
