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郎格汉斯细胞组织细胞增生症伴消化道累及。

Langerhans cell histiocytosis with digestive tract involvement.

机构信息

Pediatric Haematology and Oncology and Bone Marrow Transplantation Unit, Department of Pediatrics, Center for Child Health, Sir Ganga Ram Hospital, Delhi, India. satya

出版信息

Pediatr Blood Cancer. 2010 Oct;55(4):748-53. doi: 10.1002/pbc.22663.

DOI:10.1002/pbc.22663
PMID:20535829
Abstract

Gastrointestinal tract (GIT) involvement in Langerhans cell histiocytosis (LCH) is not commonly described. We present two children presenting with GIT involvement with LCH, one successfully treated on standard protocol and other being treated on a protocol for relapsed disease. A review of literature showed almost 95% children were less than 2 years of age and 62% were females. Vomiting, abdominal pain, constipation, intractable diarrhea, malabsorption, bloody stools, protein-losing enteropathy, and even intestinal perforation are some of the reported symptoms. More than 50% patients died within 18 months from diagnosis.

摘要

胃肠道(GIT)受累在朗格汉斯细胞组织细胞增生症(LCH)中并不常见。我们介绍了两名出现胃肠道受累的 LCH 患儿,其中一名成功地按照标准方案治疗,另一名则按照复发疾病的方案治疗。文献复习显示,近 95%的患儿年龄小于 2 岁,62%为女性。呕吐、腹痛、便秘、难治性腹泻、吸收不良、血便、蛋白丢失性肠病,甚至肠穿孔都是一些报道的症状。超过 50%的患者在诊断后 18 个月内死亡。

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