新生儿朗格汉斯细胞组织细胞增生症一例

Neonatal Langerhans cell histiocytosis:a case report

本文报告了1例患朗格汉斯细胞组织细胞增生症新生儿的临床表现、辅助检查、诊断及治疗情况。该患儿因生后发现皮疹伴反应差6h入院,入院后通过影像学及皮肤活检、免疫组织化学检查等手段明确诊断。因家长放弃治疗出院,患儿死亡。朗格汉斯细胞组织细胞增生症表现多样,临床尚无特效疗法,主要治疗措施是积极控制感染,以及必要的对症、支持治疗。肺内感染常是朗格汉斯细胞组织细胞增生症致死的原因。感染也能刺激组织细胞增生,而加重原有病变。

We reported a case of neonatal Langerhans cell histiocytosis (LCH)with his clinical manifestations,examinations,diagnosis and treatment.This infant was hospitalized due to the rash and poor response.He was diagnosed as LCH after admission by imaging,skin biopsy and immunohistochemistry.The infant died because his parents refused all possible treatments.At the moment,no effective treatment is available for LCH patients,while the main managements are infection control and necessary supportive care.Pulmonary infections often contribute to the death of LCH patient and can aggravate the situation by stimulating histiocytic hyperplasia.