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以巨脾为表现的结节病:2例无肺部受累情况。

Sarcoidosis presenting with massive splenomegaly: 2 cases without pulmonary involvement.

作者信息

Islam Md Sadiqul, Ayaz Kfm, Marisa Sumaiya Farah, Rahman Mohammad Mahfuzur, Sami Chowdhury Adnan

机构信息

Department of Internal Medicine, Evercare Hospital Dhaka, Dhaka, Bangladesh.

出版信息

Radiol Case Rep. 2025 Jul 12;20(10):4885-4889. doi: 10.1016/j.radcr.2025.06.031. eCollection 2025 Oct.

DOI:10.1016/j.radcr.2025.06.031
PMID:40843293
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12365763/
Abstract

Sarcoidosis is a chronic systemic granulomatous disease presenting in the lungs and mediastinal lymph nodes. Extrapulmonary disease alone is rare and involvement of the spleen alone, in the absence of the lung, is even rarer. Splenomeglay is unusual as a first symptom and gives a diagnostic problem, especially in areas with high prevalence of infectious or haematologic diseases. We describe 2 young adults who had massive splenomegaly, and minimal systemic symptoms, with no evidence of either clinical or radiologic thoracic sarcoidosis. Both patients were thoroughly investigated for infection, neoplasm, and autoimmune causes. Imaging demonstrated splenomegaly, multiple hypodense areas of the spleen. Splenic biopsy was not performed because of the risk of bleeding and tissue was sampled from radiologically abnormal but accessible liver and lymph node. Histological examination revealed noncaseating granulomas and the stains for acid fast bacilli and fungiwere negative. Both the patients were managed with oral corticosteroids with symptomatic relief and radiological regression of the splenic lesions without splenectomy. These cases emphasize the need for considering sarcoidosis in the differential diagnosis of splenomegaly of unknown etiology and to avoid surgical intervention like splenectomy.

摘要

结节病是一种慢性全身性肉芽肿性疾病,主要累及肺部和纵隔淋巴结。单纯肺外疾病较为罕见,而仅脾脏受累且无肺部病变的情况更为罕见。脾肿大作为首发症状并不常见,会带来诊断难题,尤其是在感染性或血液系统疾病高发地区。我们描述了2例年轻成人,他们有巨大脾肿大,全身症状轻微,无临床或放射学上的胸内结节病证据。对这两名患者都进行了关于感染、肿瘤和自身免疫病因的全面检查。影像学检查显示脾肿大,脾脏有多个低密度区。由于有出血风险,未进行脾活检,而是从放射学上异常但可触及的肝脏和淋巴结取组织样本。组织学检查发现非干酪样肉芽肿,抗酸杆菌和真菌染色均为阴性。两名患者均接受口服皮质类固醇治疗,症状缓解,脾脏病变放射学表现消退,未行脾切除术。这些病例强调了在病因不明的脾肿大鉴别诊断中需要考虑结节病,并避免像脾切除术这样的手术干预。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2695/12365763/a60ac69af570/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2695/12365763/ceebc575b482/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2695/12365763/a60ac69af570/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2695/12365763/ceebc575b482/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2695/12365763/a60ac69af570/gr2.jpg

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本文引用的文献

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Sarcoidosis presenting as isolated massive splenomegaly: A case report.以孤立性巨脾为表现的结节病:一例报告。
Clin Case Rep. 2024 Aug 6;12(8):e9283. doi: 10.1002/ccr3.9283. eCollection 2024 Aug.
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Two Cases with Isolated Splenic Sarcoidosis Diagnosed by an Ultrasound-guided Fine-needle Aspiration Biopsy.超声引导下细针穿刺活检诊断孤立性脾肉样瘤 2 例
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State-of-the-Art Treatments for Sarcoidosis.结节病的最新治疗方法。
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Hepatosplenic sarcoidosis.肝脾结节病
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Sarcoidosis and Its Splenic Wonder: A Rare Case of Isolated Splenic Sarcoidosis.结节病及其脾脏奇观:一例罕见的孤立性脾脏结节病病例
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Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999.结节病声明。美国胸科学会(ATS)、欧洲呼吸学会(ERS)和结节病及其他肉芽肿性疾病世界协会(WASOG)的联合声明,1999年2月经ATS董事会和ERS执行委员会通过。
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