成功治疗以毛细血管渗漏综合征为首发症状的噬血细胞综合征1例并文献复习

Journal of Internal Intensive Medicine(2021)

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Abstract
噬血细胞综合征(hemophagocytic syndlome,HPS)又称噬血细胞性淋巴组织细胞增生症(he-mophagocytic lymphohistiocytosis,HLH)是一组由各种因素导致淋巴细胞和组织细胞的异常激活,分泌大量细胞因子,累及全身多系统的炎症反应性疾病[1].分为原发性噬血细胞综合征(primary he-mophagocytic lymphohistiocytosis,pHLH)与继发性噬血细胞综合征(secondary hemophagocytic lymphohis-tiocytosis,sHLH),而后者的发生与感染,自身免疫性疾病,药物使用或肿瘤等多种因素有关[2].
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