Not all hemophagocytes are created equally: appreciating the heterogeneity of the hemophagocytic syndromes.
Not all hemophagocytes are created equally: appreciating the heterogeneity of the hemophagocytic syndromes.
复制标题
DOI：
10.1097/bor.0b013e32834dd37e
复制
发表时间：
2012-01
影响因子：
5.1
通讯作者：
Behrens EM
中科院分区：
文献类型：
--
作者：
Canna SW;Behrens EM
致命的巨噬细胞激活综合征（MAS）是为数不多的风湿病急症之一。 MAS 是一大类称为噬血细胞综合征的疾病的一部分，常见于感染、恶性肿瘤或遗传性免疫缺陷。由于这些疾病的临床相似性，许多临床医生倾向于在诊断标准和治疗范式上以相似的方式处理所有这些疾病。该领域的新研究表明，并非所有噬血细胞综合征都是一样的。我们将回顾人类和小鼠模型中与噬血细胞综合征（包括 MAS）的诊断、病因和治疗相关的最新文献。针对不同噬血细胞综合征的更具体的诊断标准正在制定中。动物模型表明噬血细胞综合征的产生至少有两种不同的机制：抗原呈递增强和 Toll 样受体信号传导过度。人体研究表明不同的细胞因子谱以及针对各种噬血细胞综合征的不同治疗策略。本文回顾的最新研究表明，尽管临床相似，但不同的噬血细胞综合征确实可能存在异质性。需要针对潜在疾病或遗传背景量身定制的诊断标准和治疗策略，并有望通过该领域的未来工作来解决。
The deadly Macrophage Activation Syndrome (MAS) constitutes one of the few rheumatologic emergencies. MAS is part of a larger group of diseases referred to as hemophagocytic syndromes that are seen in infections, malignancies, or genetic immunodeficiencies. Because of the clinical similarity of these diseases, many clinicians are tempted to approach them all similarly, both in diagnostic criteria and treatment paradigms. New work in the field suggests that not all hemophagocytic syndromes are equal. We will review the latest literature from both human and murine models related to the diagnosis, etiology, and treatment of hemophagocytic syndromes including MAS. More specific diagnostic criteria for the different hemophagocytic syndromes are being developed. Animal models suggest at least two different mechanisms by which hemophagocytic syndromes arise: enhanced antigen presentation and excessive Toll-like receptor signaling. Work in humans suggests different cytokine profiles, and different treatment strategies for the variety of hemophagocytic syndromes. The recent studies reviewed in this article suggest that despite clinical similarities the different hemophagocytic syndromes are indeed likely heterogeneous. Diagnostic criteria and treatment strategies tailored to the underlying disease or genetic context are needed and will hopefully be addressed by future work in this field.