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一名患有多系统朗格汉斯细胞组织细胞增多症新生儿的延迟治疗反应 病例报告及文献综述

Delayed Treatment Response in a Neonate with Multisystem Langerhans Cell Histiocytosis Case report and review of literature.

作者信息

Singh Amitabh, Mandal Anirban, Singh Lavleen, Mishra Sataroopa, Patel Ankita

机构信息

Department of Pediatrics, Chacha Nehru Bal Chikitsalya, New Delhi, India.

Department of Pediatrics, Sitaram Bhartia Institute of Science and Research, New Delhi, India.

出版信息

Sultan Qaboos Univ Med J. 2017 May;17(2):e225-e228. doi: 10.18295/squmj.2016.17.02.016. Epub 2017 Jun 20.

Abstract

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of the Langerhans cells, which are part of the mononuclear phagocytic system. The disorder varies in terms of the extent of the disease, its natural course and patient outcomes. While skin rashes are a common presentation of neonatal LCH, other systems or organs may also be involved. Delays in the diagnosis of neonatal LCH may occur due to its non-specific presentation and a lack of awareness of the condition among doctors. We report a two-month-old male neonate who presented to the Chacha Nehru Bal Chikitsalya hospital, New Delhi, India, in 2016 after the onset of pulmonary symptoms. He had been noted to have a generalised rash which had progressively worsened from 15 days of age. Following a skin biopsy and chest imaging, he was diagnosed with multisystem LCH with risk organ involvement. There was a delayed response to combined chemotherapy with no major side-effects.

摘要

朗格汉斯细胞组织细胞增多症(LCH)是一种罕见的朗格汉斯细胞增殖性疾病,朗格汉斯细胞是单核吞噬系统的一部分。该疾病在疾病范围、自然病程和患者预后方面存在差异。虽然皮疹是新生儿LCH的常见表现,但其他系统或器官也可能受累。由于其表现不具特异性且医生对该疾病认识不足,新生儿LCH可能会出现诊断延迟。我们报告一例2016年出现肺部症状后就诊于印度新德里查查·尼赫鲁儿童医院的两个月大男婴。自15日龄起,他被发现有全身性皮疹,且皮疹逐渐加重。经过皮肤活检和胸部影像学检查,他被诊断为多系统LCH并伴有风险器官受累。联合化疗反应延迟,且无重大副作用。

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本文引用的文献

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Langerhans cell histiocytosis in neonates.新生儿朗格汉斯细胞组织细胞增多症
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