Suppr超能文献

病例报告:无多发性神经病的非典型POEMS综合征,并发边缘性TAFRO综合征。

Case report: Atypical POEMS syndrome without polyneuropathy, complicated by borderline TAFRO syndrome.

作者信息

Tan Shuai, Shang Mingyue, Masaki Yasufumi, Ni Jing, Da Yuwei, Sun Jing, Cao Yaofang, Su Li, Sun Wanling

机构信息

Department of Hematology, Xuanwu Hospital, Capital Medical University, Beijing, China.

Hematology and Immunology, Kanazawa Medicai University, Uchinada, Japan.

出版信息

Front Med (Lausanne). 2024 Dec 10;11:1445971. doi: 10.3389/fmed.2024.1445971. eCollection 2024.

Abstract

POEMS syndrome is a complex and rare hematological disease involving multiple physiological systems. According to the currently accepted diagnostic criteria for POEMS syndrome, polyneuropathy is one of the primary mandatory criteria. A patient presented with M protein, Castleman disease (CD), elevated vascular endothelial growth factor (VEGF), extravascular volume overload, and endocrinopathy. However, edema of the extremities hindered the diagnosis of polyneuropathy by electromyography (EMG). Eventually, we diagnosed the patient with atypical POEMS syndrome without polyneuropathy. The case also exhibited features consistent with TAFRO syndrome, such as anasarca, thrombocytopenia, and renal insufficiency. This underscores the need to emphasize that TAFRO syndrome is principally a systemic inflammatory disorder. Timely diagnosis and treatment with dexamethasone, followed by several sessions of lenalidomide and dexamethasone (Rd) regimen chemotherapy, resulted in complete remission (CR), and was followed by autologous stem cell transplantation (ASCT). This case offers valuable insights into the diagnosis and treatment of POEMS syndrome, which may prompt a reconsideration of the diagnostic criteria for this syndrome.

摘要

POEMS综合征是一种累及多个生理系统的复杂罕见血液疾病。根据目前公认的POEMS综合征诊断标准,多发性神经病是主要的必备标准之一。一名患者出现M蛋白、卡斯特曼病(CD)、血管内皮生长因子(VEGF)升高、血管外容量超负荷和内分泌病。然而,肢体水肿妨碍了通过肌电图(EMG)诊断多发性神经病。最终,我们诊断该患者为无多发性神经病的非典型POEMS综合征。该病例还表现出与TAFRO综合征一致的特征,如全身性水肿、血小板减少和肾功能不全。这凸显了强调TAFRO综合征主要是一种全身性炎症性疾病的必要性。及时用地塞米松进行诊断和治疗,随后进行几个疗程的来那度胺和地塞米松(Rd)方案化疗,导致完全缓解(CR),随后进行自体干细胞移植(ASCT)。该病例为POEMS综合征的诊断和治疗提供了宝贵的见解,可能促使重新考虑该综合征的诊断标准。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ef0e/11666388/b88e37161133/fmed-11-1445971-g001.jpg

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验