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以孤立性巨脾为表现的结节病:一例报告。

Sarcoidosis presenting as isolated massive splenomegaly: A case report.

作者信息

Kyriakos Saad Melissa, El Hajj Imad, Saikaly Elias

机构信息

Saint George Hospital University Medical Center Beirut Lebanon.

Department of General Surgery Saint George Hospital University Medical Center, Saint George University of Beirut Beirut Lebanon.

出版信息

Clin Case Rep. 2024 Aug 6;12(8):e9283. doi: 10.1002/ccr3.9283. eCollection 2024 Aug.

DOI:10.1002/ccr3.9283
PMID:39114850
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11303656/
Abstract

KEY CLINICAL MESSAGE

Sarcoidosis is a systemic granulomatous disease with an unknown cause, marked by the presence of noncaseating granulomas in the affected organs. While the pulmonary interstitium is most frequently involved, the disease can affect almost any other organ system. Extrapulmonary involvement can occur with or without lung involvement, but isolated extrapulmonary involvement is a rare event. Isolated splenomegaly is very rare and presents an uncommon manifestation of sarcoidosis, its diagnoses is a challenge due to a broad differential diagnosis. Here, we present an intriguing case of a 28-year-old male with isolated splenic sarcoidosis.

ABSTRACT

Sarcoidosis is a systemic disease of unknown cause, marked by the presence of noncaseating granulomas in affected organs. It most frequently impacts the pulmonary interstitium, though it can also affect nearly any other organ system. This involvement can occur with or without lung involvement, but isolated extrapulmonary cases are observed in only about 10% of instances. Furthermore, isolated splenomegaly is an exceptionally rare event and an uncommon presentation of sarcoidosis, posing a significant clinical challenge due to the wide differential diagnosis. Potential differential diagnoses include hematologic cancers, primary or metastatic splenic tumors, infiltrative diseases, inflammatory conditions, and infections. We present a noteworthy case of a 28-year-old with isolated splenic sarcoidosis.

摘要

关键临床信息

结节病是一种病因不明的全身性肉芽肿性疾病,其特征是在受影响器官中存在非干酪样肉芽肿。虽然肺间质最常受累,但该疾病几乎可影响任何其他器官系统。肺外受累可在有或无肺部受累的情况下发生,但孤立性肺外受累是罕见事件。孤立性脾肿大非常罕见,是结节病的一种不常见表现,由于鉴别诊断范围广泛,其诊断具有挑战性。在此,我们报告一例28岁男性孤立性脾脏结节病的有趣病例。

摘要

结节病是一种病因不明的全身性疾病,其特征是在受影响器官中存在非干酪样肉芽肿。它最常影响肺间质,不过也可影响几乎任何其他器官系统。这种受累可在有或无肺部受累的情况下发生,但仅约10%的病例为孤立性肺外病例。此外,孤立性脾肿大是一种极其罕见的事件,也是结节病的一种不常见表现,由于鉴别诊断范围广泛,构成重大临床挑战。潜在的鉴别诊断包括血液系统癌症、原发性或转移性脾脏肿瘤、浸润性疾病、炎症性疾病和感染。我们报告一例28岁孤立性脾脏结节病的值得关注的病例。

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引用本文的文献

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mTORC1-Dependent Regulation of the CCL24-CCR3 Axis Controls Granuloma Formation and Maintenance in Sarcoidosis.mTORC1依赖性对CCL24-CCR3轴的调控控制结节病中的肉芽肿形成和维持。
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本文引用的文献

1
Angiomyxolipoma of the spermatic cord: A case report.精索血管平滑肌脂肪瘤:一例报告。
Clin Case Rep. 2024 May 24;12(6):e8971. doi: 10.1002/ccr3.8971. eCollection 2024 Jun.
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New trends in ultrasound of hepatosplenic sarcoidosis.肝脾结节病的超声新趋势。
Z Gastroenterol. 2015 Apr;53(4):283-4. doi: 10.1055/s-0034-1399122. Epub 2015 Apr 10.
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Pulmonary manifestations of sarcoidosis.结节病的肺部表现。
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Atypical sarcoidosis: case reports and review of the literature.非典型结节病:病例报告及文献综述
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Hepatic, splenic, and gastrointestinal involvement with sarcoidosis.结节病累及肝脏、脾脏和胃肠道。
Semin Respir Crit Care Med. 2002 Dec;23(6):529-41. doi: 10.1055/s-2002-36517.
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Laparoscopic splenectomy for isolated sarcoidosis of the spleen.腹腔镜下脾切除术治疗孤立性脾结节病。
J Laparoendosc Adv Surg Tech A. 2005 Apr;15(2):160-2. doi: 10.1089/lap.2005.15.160.
10
Imaging manifestations of abdominal sarcoidosis.腹部结节病的影像学表现。
AJR Am J Roentgenol. 2004 Jan;182(1):15-28. doi: 10.2214/ajr.182.1.1820015.