Hemophagocytic syndrome (HPS) in children and adults.
Hemophagocytic syndrome (HPS) in children and adults.
复制标题
儿童和成人的噬血细胞综合征（HPS）。
DOI：
10.1016/s0925-5710(96)00560-9
复制
发表时间：
1997
影响因子：
2.1
通讯作者：
H. Tsuda
中科院分区：
文献类型：
--
作者：
H. Tsuda
噬血细胞综合征（HPS）是一种以单核细胞-巨噬细胞-组织细胞谱系的良性噬血细胞的全身性增殖为特征的临床病理实体，与发热、血细胞减少、肝脾肿大、淋巴结病和凝血病相关。两种形式的综合征已得到很好的表征：家族性噬血细胞性淋巴组织细胞增多症（FHL）的婴儿和反应性噬血细胞综合征（RHS）遇到在任何年龄。虽然HPS的临床特征和病理生理学已被报道，但儿童和成人HPS临床特征的差异尚未得到广泛研究。本文对HPS的最新概念和临床特征进行综述，重点介绍不同发病年龄HPS的临床特征。
Hemophagocytic'syndrome (HPS) is a clinicopathologic entity characterized by systemic proliferation of benign hemophagocytic cells of the monocyte-macrophage-histiocyte lineage, associated with fever, cytopenias, hepatosplenomegaly, lymphadenopathy, and coagulopathy. Two forms of the syndrome have been well characterized; familial hemophagocytic lymphohistiocytosis (FHL) of infants and reactive hemophagocytic syndrome (RHS) encountered at any age. Although the clinical features and pathophysiology of HPS have been reported, the differences in the clinical characteristics of HPS in children and adults have not been studied extensively. In this article the latest concepts and clinical features of HPS are reviewed, focusing on the differences in features depending on the age of onset.