Diagnostic Challenges of Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin Changes (POEMS) Syndrome: A Rare Case Report and Review of the Literature.

Kailash Kumar, Rohit Daga, Jitendra Singh, Nilesh Kumar, Anju Dinkar
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Abstract

Introduction: POEMS syndrome is a rare multisystem disorder associated with plasma cell dyscrasia and abnormal cytokine production, including vascular endothelial growth factor (VEGF). The mandatory criterion for its diagnosis includes polyneuropathy and monoclonal plasma cell disorder, along with other major and minor criteria. This case highlights the diagnostic and therapeutic challenges of POEMS syndrome by depicting the case of a 61-year-old male with progressive sensory-motor polyneuropathy, lymphadenopathy, and splenomegaly.

Case presentation: The patient presented with a year-long history of bilateral limb weakness and sensory disturbances, accompanied by abdominal distention, weight loss, and other systemic symptoms. Clinical examination revealed skin hyperpigmentation, splenomegaly, and a right axillary lymph node enlargement. Neurological evaluation showed distal limb hypotonia, absent reflexes, and sensory deficits. Diagnostic investigations, including nerve conduction studies, imaging, and bone marrow biopsy, confirmed POEMS syndrome based on polyneuropathy, monoclonal IgG lambda plasma cells, Castleman disease, sclerotic bone lesions, elevated VEGF, and minor criteria, such as endocrinopathy and skin changes. The treatment comprised lenalidomide and dexamethasone, resulting in significant improvement at the three-month follow-up, including normalized VEGF levels and resolution of ascites.

Conclusion: This case highlights the necessity of identifying the many presentations of POEMS syndrome for prompt diagnosis and treatment. Despite its rarity and diagnostic complexity, prompt treatment can significantly improve clinical outcomes. POEMS syndrome should be considered in patients with unexplained neuropathy and systemic features, enabling better outcomes through targeted therapies.

多神经病变、器官肿大、内分泌病变、m蛋白和皮肤变化(POEMS)综合征的诊断挑战:罕见病例报告和文献回顾。
简介:POEMS综合征是一种罕见的多系统疾病,与浆细胞病变和细胞因子产生异常有关,包括血管内皮生长因子(VEGF)。其诊断的强制性标准包括多神经病变和单克隆浆细胞障碍,以及其他主要和次要标准。本病例通过描述一名61岁男性进行性感觉-运动多神经病变、淋巴结病和脾肿大的病例,突出了POEMS综合征的诊断和治疗挑战。病例表现:患者有一年的双侧肢体无力和感觉障碍病史,并伴有腹胀、体重减轻和其他全身症状。临床检查发现皮肤色素沉着,脾肿大,右腋窝淋巴结肿大。神经学评估显示远端肢体张力低下,反射缺失和感觉缺陷。诊断调查,包括神经传导研究、影像学和骨髓活检,证实POEMS综合征基于多神经病变、单克隆IgG lambda浆细胞、Castleman病、硬化性骨病变、VEGF升高和次要标准,如内分泌病变和皮肤改变。治疗包括来那度胺和地塞米松,在三个月的随访中显著改善,包括VEGF水平正常化和腹水消退。结论:本病例强调了识别POEMS综合征的多种表现以及时诊断和治疗的必要性。尽管罕见且诊断复杂,但及时治疗可显著改善临床结果。有不明原因的神经病变和全身性特征的患者应考虑POEMS综合征,通过靶向治疗获得更好的结果。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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