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噬血细胞性淋巴组织细胞增生症的影像学:一篇全面的图谱文章。

Imaging in Hemophagocytic Lymphohistiocytosis: A Comprehensive Pictorial Essay.

作者信息

Bhatnagar Caleb, Allam Emad, Illimoottil Mathew, Illimoottil Sarah, Errampalli Eric, Kosaraju Sriya

机构信息

Radiology, Loyola University Medical Center, Maywood, USA.

Radiology, University of Missouri Kansas City School of Medicine, Kansas City, USA.

出版信息

Cureus. 2025 Aug 1;17(8):e89203. doi: 10.7759/cureus.89203. eCollection 2025 Aug.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening condition characterized by excessive immune activation, resulting in severe systemic inflammation and multiorgan failure. This pictorial essay presents an overview of HLH imaging findings, accompanied by a discussion of pathophysiology, diagnosis, and treatment approaches. Imaging is crucial in diagnosing and monitoring HLH by identifying key features, such as splenomegaly, lymphadenopathy, and central nervous system (CNS) involvement. While the imaging findings associated with HLH are nonspecific and do not constitute part of the diagnostic criteria for HLH, they are helpful in the diagnostic process and can offer valuable insights into the extent of organ involvement and disease progression, guiding timely interventions. This article emphasizes the importance of recognizing imaging patterns associated with HLH for early diagnosis and effective management.

摘要

噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见但危及生命的疾病,其特征是免疫过度激活,导致严重的全身炎症和多器官功能衰竭。这篇图文并茂的文章概述了HLH的影像学表现,并讨论了其病理生理学、诊断和治疗方法。影像学对于HLH的诊断和监测至关重要,通过识别关键特征,如脾肿大、淋巴结病和中枢神经系统(CNS)受累情况。虽然与HLH相关的影像学表现是非特异性的,且不构成HLH诊断标准的一部分,但它们在诊断过程中很有帮助,并且可以提供有关器官受累程度和疾病进展的有价值见解,从而指导及时干预。本文强调认识与HLH相关的影像学模式对于早期诊断和有效管理的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2cfe/12399025/b8956660ecfe/cureus-0017-00000089203-i01.jpg

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