特发性多中心Castleman病相关POEMS综合征1例报告

Amritha Radhakrishnan Nair, Changaramkumarath Gichin, Deepa Pramod Raj Santhi, Moothiringode Chithrabhanu Savithri
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摘要

Castleman病是一种罕见的特发性淋巴细胞增生性疾病,由Benjamin Castleman博士于1954年首次发现。在临床实践中是一种相当罕见的疾病。然而,发病率不容忽视。它可能影响单个淋巴结(单中心性CD)或整个身体的多个淋巴结(多中心性CD)。管理取决于疾病的类型和严重程度以及病情的早期识别。我们的病例报告是一名60岁的女性患者,其潜在的合并症包括2型糖尿病,甲状腺功能减退,其主诉为全身无力,体重减轻,脱发,无法解决的粘液化脓性鼻漏,双侧足部水肿和左侧多发,非压痛,坚硬和移动的I级和II级AJCC颈椎淋巴结病变,>2cm,后来活检并诊断为Castleman病相关POEMS综合征。本报告的目的是考虑将乳糜泻作为具有相似临床表现的患者的鉴别诊断,早期识别和治疗可能提供更好的预后。我们还想强调3个要点:1)组织学在将其与其他淋巴增生性疾病区分开来方面的意义。2)即使在MCD中,切除麻烦的LN也可以诊断并在一定程度上被认为是治疗性的。类固醇治疗对控制病情进展有有益作用。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A Case Report on Idiopathic-Multi-centric Castleman’s Disease Associated POEMS- Syndrome
: Castleman’s Disease is a rare lymphoproliferative disorder of idiopathic etio-pathogenesis and was first described by Dr Benjamin Castleman in 1954. It is a rather rare disease entity in clinical practice. However, the incidence cannot be ignored. It may affect a single lymph node (Unicentric CD) or multiple lymph nodes throughout the body (Multicentric CD). Management depends upon the type and severity of the disease and early recognition of the condition. Our case report is that of a 60-year-old female patient with underlying comorbidities including type 2 diabetes mellitus, hypothyroidism who presented with complaints of generalized weakness, weight loss, hair loss, non-resolving muco-purulent rhinorrhea, bilateral pedal edema and multiple, non-tender, firm and mobile left sided Level I and II ‘AJCC’ cervical lymphadenopathy of >2cm which was later biopsied and diagnosed to be Castleman’s disease associated POEMS syndrome. The aim of this report is to consider CD as a differential diagnosis in patients presenting with similar clinical picture and early identification and treatment may provide a better prognosis. We would also like to highlight 3 take home points: 1) The significance of histology in differentiating it from other lymph proliferative conditions. 2) LN excision of the bothersome LN can be diagnostic and may be considered therapeutic to some extend even in MCD. 3) Steroid therapy has beneficial effects to control progression of the disease.
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