目的：探讨新生儿噬血细胞综合征的临床特点（hemophagocytic lymphohistiocytosis），提高对该病的认识。 方法：回顾性分析1例-早产儿噬血细胞综合征合并坏死性小肠结肠炎，并以“ 淋巴组织细胞增多症”，“ 噬血细胞综合征”，“新生儿”，“早产儿”，”hemophagocytic lymphohistiocytosis","premature","neonatal"，“necrotizing enterocolitis”等为关键词，在万方数据库，Pubmed,中国期刊全文数据库，Web of science上从建库至今进行文献检索，结合本病例的诊疗，总结HLH的临床特点。 结果 参照2004HLH的诊断标准，本病例HLH诊断明确。激素治疗过程中出现了坏死性小肠结肠炎，暂停激素待坏死性小肠结肠炎缓解后继续激素治疗，该病例治愈出院，未造成肠穿孔并发症。 结论 早期诊断，早期干预，密切监护，有助于改善预后。治疗不能照搬2004HLH国际指南，早产儿HLH的治疗方案仍需进一步的探讨。
Objective: To investigate the clinical features of neonatal hemophagocytic syndrome (HPCS) and improve the understanding of the disease. Methods: a case of hemophagocytic syndrome of premature infant with necrotizing enterocolitis was analyzed retrospectively. The keywords were "lymphohistiocytosis", "hemophagocytic syndrome", "newborn", "premature infant", "hemophagocytic lymphohistiocytosis", "prognosis", "neonatal" and "necrotizing enterocolitis", The clinical characteristics of HLH were summarized based on the diagnosis and treatment of this case. Results according to the diagnostic criteria of 2004 HLH, the diagnosis of HLH was clear. Necrotizing enterocolitis occurred in the course of hormone treatment. Hormone therapy was suspended until necrotizing enterocolitis was relieved. The patient was cured and discharged without intestinal perforation. Conclusion early diagnosis, early intervention and close monitoring are helpful to improve the prognosis. However, the treatment of HLH in preterm infants should be further explored.