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噬血细胞性淋巴组织细胞增生症诊断之迷宫:6例临床病例的单中心经验

The Maze of Diagnosing Hemophagocytic Lymphohistiocytosis: Single-Center Experience of a Series of 6 Clinical Cases.

作者信息

Hanoun Maher, Dührsen Ulrich

机构信息

Department of Hematology, University Hospital, University of Duisburg-Essen, Essen, Germany.

出版信息

Oncology. 2017;92(3):173-178. doi: 10.1159/000454733. Epub 2017 Jan 5.

Abstract

Clinical symptoms of hemophagocytic lymphohistiocytosis (HLH) are based on an excessive inflammatory response. Not only the diversity of the putative underlying etiologies of this rare syndrome but also the subsequent large variety of symptoms complicate the diagnosis of HLH in adult patients. However, early diagnosis and immediate treatment initiation are imperative for clinical outcome. In this article, we will review the diagnostic criteria of HLH and, in this context, discuss 6 cases, each of whom presented with a different clinical appearance causally associated with distinct malignant and nonmalignant diseases, exemplifying the spectrum of associations and manifestations of HLH.

摘要

噬血细胞性淋巴组织细胞增生症(HLH)的临床症状基于过度的炎症反应。这种罕见综合征潜在病因的多样性以及随之而来的各种各样的症状,使成年患者HLH的诊断变得复杂。然而,早期诊断和立即开始治疗对于临床结果至关重要。在本文中,我们将回顾HLH的诊断标准,并在此背景下讨论6例病例,每例病例都呈现出与不同的恶性和非恶性疾病因果相关的不同临床表现,例证了HLH的关联和表现范围。

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