• Users Online: 798
  • Print this page
  • Email this page
Year : 2023  |  Volume : 3  |  Issue : 2  |  Page : 81-85

Isolated Hepatobiliary Involvement in an Infant with Langerhans Cell Histiocytosis: A Case Report with Review of Literature

1 Department of Pediatric Medicine, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India
2 Department of GI and Hepatobiliary Pathology, School of Digestive and Liver Disease, Kolkata, West Bengal, India

Correspondence Address:
Dr. Niva Lakra
Bhatia Basti, Main Road, Kadma, Jamshedpur, Jharkhand
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ipcares.ipcares_18_23

Rights and Permissions

Background: Langerhans cell histiocytosis (LCH) is a rare condition in which there is the proliferation of cells of the monocyte-dendritic cell lineage, including Langerhan's cells, with variable clinical manifestations and outcomes. Hepatic involvement is often seen as a part of multi-system LCH and is associated with a fulminant course. Isolated hepatobiliary involvement in LCH is rare. Clinical Description: We report an 11-month-old female infant presenting with prolonged fever associated with progressive jaundice, clay-colored stools, and a massive hepatomegaly. Liver biopsy showed CD1a and Langerin positivity. There was no other system involvement, and hence, the infant was diagnosed with a case of single-system LCH with isolated hepato-biliary LCH. Management and Outcome: A customized chemotherapeutic regimen was started with cytarabine and prednisolone. The patient has responded and there has been gradual improvement in the general clinical condition as well as the laboratory parameters. Conclusions: This case report creates the awareness of possible presentation of pediatric LCH with isolated liver involvement. A high index of suspicion, carefully customized chemotherapeutic regimen may help in the timely diagnosis and successful management.

Print this article     Email this article
 Next article
 Previous article
 Table of Contents

 Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
 Citation Manager
 Access Statistics
 Reader Comments
 Email Alert *
 Add to My List *
 * Requires registration (Free)

 Article Access Statistics
    PDF Downloaded24    
    Comments [Add]    

Recommend this journal