以腹泻为主要症状的POEMS综合征复发患者:一例病例报告
Recurrent POEMS Syndrome Presenting with Diarrhea as the Dominant Symptom: A Case Report
摘要: POEMS综合征是一种罕见的单克隆浆细胞疾病,其临床特征为多发性神经病、脏器肿大、内分泌病变、单克隆免疫球蛋白血症以及皮肤改变。腹泻症状在POEMS综合征中并不常见。我们报告了一例以腹泻为主要表现的复发性POEMS综合征患者的病例。该患者接受了多次胃镜检查和息肉切除术,但症状并未改善。随后的免疫固定电泳显示存在IgA-λ型M蛋白条带,且血管内皮生长因子升高,提示可能存在POEMS综合征复发。腹泻作为POEMS综合征罕见且不典型的症状,给POEMS综合征复发的诊断带来了重大挑战。全面评估和管理对于POEMS综合征及早诊断及改善预后具有重要作用。
Abstract: POEMS syndrome is a rare monoclonal plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Diarrhea is an uncommon manifestation in POEMS syndrome. We report a case of recurrent POEMS syndrome presenting predominantly with diarrhea. The patient underwent multiple gastroscopies and polypectomies without symptomatic improvement. Subsequent immunofixation electrophoresis revealed an IgA-λ monoclonal protein band, and elevated vascular endothelial growth factor (VEGF) levels suggested potential disease recurrence. Diarrhea, as a rare and atypical symptom of POEMS syndrome, posed significant diagnostic challenges in identifying disease relapse. Comprehensive evaluation and management are critical for early diagnosis and improving prognosis in POEMS syndrome.
文章引用:张江梅, 俞坤武, 曾秋棠. 以腹泻为主要症状的POEMS综合征复发患者:一例病例报告[J]. 临床医学进展, 2025, 15(4): 665-670. https://doi.org/10.12677/acm.2025.154980

参考文献

[1] Bhattacharjee, S., Siyad, I. and Maramattom, B. (2022) Chronic Diarrhea—The Poetic Masquerade. Journal of Postgraduate Medicine, 68, 239-242. [Google Scholar] [CrossRef] [PubMed]
[2] Li, J. and Zhou, D. (2013) New Advances in the Diagnosis and Treatment of POEMS Syndrome. British Journal of Haematology, 161, 303-315. [Google Scholar] [CrossRef] [PubMed]
[3] Dispenzieri, A. (2021) POEMS Syndrome: 2021 Update on Diagnosis, Risk‐Stratification, and Management. American Journal of Hematology, 96, 872-888. [Google Scholar] [CrossRef] [PubMed]
[4] Li, J., Zhang, W., Jiao, L., Duan, M., Guan, H., Zhu, W., et al. (2011) Combination of Melphalan and Dexamethasone for Patients with Newly Diagnosed POEMS Syndrome. Blood, 117, 6445-6449. [Google Scholar] [CrossRef] [PubMed]
[5] Kourelis, T.V., Buadi, F.K., Kumar, S.K., Gertz, M.A., Lacy, M.Q., Dingli, D., et al. (2016) Long‐Term Outcome of Patients with POEMS Syndrome: An Update of the Mayo Clinic Experience. American Journal of Hematology, 91, 585-589. [Google Scholar] [CrossRef] [PubMed]
[6] Nozza, A., Terenghi, F., Gallia, F., Adami, F., Briani, C., Merlini, G., et al. (2017) Lenalidomide and Dexamethasone in Patients with poems Syndrome: Results of a Prospective, Open‐label Trial. British Journal of Haematology, 179, 748-755. [Google Scholar] [CrossRef] [PubMed]
[7] Misawa, S., Sato, Y., Katayama, K., Nagashima, K., Aoyagi, R., Sekiguchi, Y., et al. (2016) Safety and Efficacy of Thalidomide in Patients with POEMS Syndrome: A Multicentre, Randomised, Double-Blind, Placebo-Controlled Trial. The Lancet Neurology, 15, 1129-1137. [Google Scholar] [CrossRef] [PubMed]
[8] Giglia, F., Chiapparini, L., Fariselli, L., Barbui, T., Ciano, C., Scarlato, M., et al. (2007) POEMS Syndrome: Relapse after Successful Autologous Peripheral Blood Stem Cell Transplantation. Neuromuscular Disorders, 17, 980-982. [Google Scholar] [CrossRef] [PubMed]
[9] Vaz, J.A., Frada, L., Soares, M.M., Mello, E. and Silva, A. (2020) POEMS Syndrome: An Atypical Presentation with Chronic Diarrhoea and Asthenia. European Journal of Case Reports in Internal Medicine, 7, Article ID: 001241.
[10] Thakral, S., Issa, N.P., Barboi, A.C. and Lee, J.M. (2016) Prominent Dysautonomia in a Patient with POEMS Syndrome. Clinical Autonomic Research, 26, 223-228. [Google Scholar] [CrossRef] [PubMed]
[11] Fujinuma, Y., Asahina, M., Fukushima, T., Katagiri, A., Yamanaka, Y., Misawa, S., et al. (2012) Preserved Autonomic Function in Patients with POEMS Syndrome. Journal of the Neurological Sciences, 318, 131-134. [Google Scholar] [CrossRef] [PubMed]
[12] Kihara, Y., Hori, H., Murakami, H., Hatakeyama, Y., Yoshikawa, I., Hamada, T., et al. (2002) A Case of POEMS Syndrome Associated with Reactive Amyloidosis and Waldenström’s Macroglobulinaemia. Journal of Internal Medicine, 252, 255-258. [Google Scholar] [CrossRef] [PubMed]
[13] Kuwabara, S., Suichi, T. and Misawa, S. (2020) ‘Early VEGF Testing in Inflammatory Neuropathy Avoids POEMS Syndrome Misdiagnosis and Associated Costs’ by Marsh et al. Journal of Neurology, Neurosurgery & Psychiatry, 92, 118-119. [Google Scholar] [CrossRef] [PubMed]
[14] Watanabe, O., Arimura, K., Kitajima, I., Osame, M. and Maruyama, I. (1996) Greatly Raised Vascular Endothelial Growth Factor (VEGF) in POEMS Syndrome. The Lancet, 347, 702. [Google Scholar] [CrossRef] [PubMed]
[15] Imai, N., Taguchi, J., Yagi, N., Konishi, T., Serizawa, M. and Kobari, M. (2009) Relapse of Polyneuropathy, Organomegaly, Endocrinopathy, M-Protein, and Skin Changes (POEMS) Syndrome without Increased Level of Vascular Endothelial Growth Factor Following Successful Autologous Peripheral Blood Stem Cell Transplantation. Neuromuscular Disorders, 19, 363-365. [Google Scholar] [CrossRef] [PubMed]