Literature DB >> 26136948

Langerhans cell histiocytosis with multisystem involvement in an infant: A case report.

Lintao Bi1, Butong Sun1, Zhenxia Lu1, Zhangzhen Shi1, Dan Wang1, Zhenxing Zhu1.   

Abstract

Langerhans cell histiocytosis (LCH) is a proliferative disease of histiocyte-like cells, with a wide range of clinical presentations that vary from a solitary lesion to more severe multifocal or disseminated lesions. The disease can affect any age group; however, the peak incidence rate is in infants aged between 1 and 3 years-old. Diagnosis of LCH should be based on the synthetical analysis of clinical presentations, in addition to features of imaging and histopathology. Although certain cases regress spontaneously, other patients require systemic chemotherapy together with the administration of steroids. The present study reports the case of an infant with LDH with multisystem involvement, including that of the bone, skin, orbit, spleen and lungs. The patient received chemotherapy and obtained rapid improvement in the involved systems. A total of 2.5 years after completion of the therapy, the patient still remains in follow-up and no evidence of active disease has been noted.

Entities:  

Keywords:  Langerhans cell histiocytosis; multisystem involvement

Year:  2015        PMID: 26136948      PMCID: PMC4473648          DOI: 10.3892/etm.2015.2396

Source DB:  PubMed          Journal:  Exp Ther Med        ISSN: 1792-0981            Impact factor:   2.447


  30 in total

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Review 7.  Langerin/CD207 sheds light on formation of birbeck granules and their possible function in Langerhans cells.

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10.  Aberrant chemokine receptor expression and chemokine production by Langerhans cells underlies the pathogenesis of Langerhans cell histiocytosis.

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Journal:  J Exp Med       Date:  2003-05-12       Impact factor: 14.307

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  2 in total

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2.  Integrated imaging of systemic Langerhans cell histiocytosis in an infant.

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