Zarrini Pari, Mosayebi Ziba, Ramyar Asghar, Dalili Hosein
Department of Pediatrics, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
Department of Pediatrics, Breast Feeding Research Center, Tehran University of Medical Sciences, Tehran, Iran.
Acta Med Iran. 2017 Jan;55(1):82-84.
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation, occurring as either a familial disorder or a sporadic condition, in association with a variety of triggers. This article will introduce a neonate with HLH in Iran. We report a case of HLH presenting with respiratory distress and fever, hepatosplenomegaly, jaundice and pancytopenia on the second day of life. Typical clinical and laboratory findings were detected in the neonate. HLH was diagnosed according to HLH-2004 guidelines. In spite of initiating the treatment, the disease did not cure. Post-mortem, extensive hemophagocytosis was found in multiple organs. No specific genetic defect was identified. Since HLH is a potentially lethal childhood illness, early diagnosis of this disorder and commences the therapy is important for pediatricians.
噬血细胞性淋巴组织细胞增生症（HLH）是一种病理性免疫激活综合征，可作为家族性疾病或散发性疾病出现，并与多种触发因素相关。本文将介绍一名伊朗的HLH新生儿病例。我们报告一例HLH患儿，出生第二天出现呼吸窘迫、发热、肝脾肿大、黄疸和全血细胞减少。在该新生儿中检测到典型的临床和实验室检查结果。根据HLH-2004指南诊断为HLH。尽管开始了治疗，但疾病并未治愈。尸检时，在多个器官发现广泛的噬血细胞现象。未发现特定的基因缺陷。由于HLH是一种潜在致命的儿童疾病，早期诊断并开始治疗对儿科医生很重要。
