Tsuda H
Division of Clinical Hematology and Immunology, Kumamoto City Hospital, Japan.
Int J Hematol. 1997 Apr;65(3):215-26. doi: 10.1016/s0925-5710(96)00560-9.
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.
噬血细胞综合征（HPS）是一种临床病理实体，其特征为单核细胞-巨噬细胞-组织细胞谱系的良性噬血细胞发生全身性增殖，并伴有发热、血细胞减少、肝脾肿大、淋巴结病和凝血病。该综合征的两种形式已得到充分描述；婴儿的家族性噬血细胞性淋巴组织细胞增生症（FHL）和任何年龄都可能出现的反应性噬血细胞综合征（RHS）。尽管已有关于HPS临床特征和病理生理学的报道，但儿童和成人HPS临床特征的差异尚未得到广泛研究。本文综述了HPS的最新概念和临床特征，重点关注发病年龄不同导致的特征差异。
