Multisystem Langerhans cell histiocytosis with liver dysfunction as the first presentation: A case report
- Authors:
- Dian-Gang Liu
- Yu-Xian Zhang
- Fei Li
View Affiliations
Affiliations: Department of General Surgery, Xuanwu Hospital, Capital Medical University, Xuanwu, Beijing 100053, P.R. China, Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing 100053, P.R. China
- Published online on: October 26, 2011 https://doi.org/10.3892/ol.2011.462
-
Pages:
391-394
Metrics: Total
Views: 0 (Spandidos Publications: | PMC Statistics: )
Metrics: Total PDF Downloads: 0 (Spandidos Publications: | PMC Statistics: )
This article is mentioned in:
Abstract
Langerhans cell histiocytosis (LCH) is a rare disease of unknown etiology characterized by oligoclonal proliferation of Langerhans cells. The diagnosis of LCH is complicated by the fact that it may involve multiple organ systems and its clinical presentation and course varies, ranging from an isolated to a multisystem disease. We report a 35-year‑old male with LCH involving multiple systems, including the bones, lungs, spleen, liver and bile ducts, whose first clinical presentation was liver dysfunction. The patient was diagnosed following a skull biopsy that revealed infiltration of Langerhans cells. However, a liver biopsy revealed sclerosing cholangitis (SC) with no signs of Langerhans cell infiltration, and the clinical manifestations of the involved organs were atypical, leading to a delayed diagnosis. The patient was in partial remission following chemotherapy. In conclusion, findings of this case may aid our understanding of the pathophysiology of LCH and in improving its diagnosis and treatment.
View References
|
1
|
Yağci B, Varan A, Cağlar M, Söylemezoğlu
F, Sungur A, Orhan D, Yalçin B, Akyüz C, Kutluk T and Büyükpamukçu
M: Langerhans cell histiocytosis: retrospective analysis of 217
cases in a single center. Pediatr Hematol Oncol. 25:399–408.
2008.PubMed/NCBI
|
|
2
|
Lau SK, Chu PG and Weiss LM:
Immunohistochemical expression of langerin in Langerhans cell
histiocytosis and non-Langerhans cell histiocytic disorders. Am J
Surg Pathol. 32:615–619. 2008. View Article : Google Scholar : PubMed/NCBI
|
|
3
|
Abla O, Egeler RM and Weitzman S:
Langerhans cell histiocytosis: current concepts and treatments.
Cancer Treat Rev. 36:354–359. 2010. View Article : Google Scholar : PubMed/NCBI
|
|
4
|
Stockschlaeder M and Sucker C: Adult
Langerhans cell histiocytosis. Eur J Haematol. 76:363–368. 2006.
View Article : Google Scholar
|
|
5
|
Satter EK and High WA: Langerhans cell
histiocytosis: a review of the current recommendations of the
Histiocyte Society. Pediatr Dermatol. 25:291–295. 2008. View Article : Google Scholar : PubMed/NCBI
|
|
6
|
Weismüller TJ, Wedemeyer J, Kubicka S,
Strassburg CP and Manns MP: The challenges in primary sclerosing
cholangitis-aetiopathogenesis, autoimmunity, management and
malignancy. J Hepatol. 48:S38–S57. 2008.PubMed/NCBI
|
|
7
|
Wong A, Ortiz-Neira CL, Reslan WA, Sharon
R, Pinto-Rojas A, Kaura D and Anderson R: Liver involvement in
Langerhans cell histiocytosis. Pediatr Radiol. 36:1105–1107. 2006.
View Article : Google Scholar : PubMed/NCBI
|
|
8
|
Schmidt S, Eich G, Hanquinet S,
Tschäppeler H, Waibel P and Gudinchet F: Extra-osseous involvement
of Langerhans’ cell histiocytosis in children. Pediatr Radiol.
34:313–321. 2004.
|
|
9
|
Windebank K and Nanduri V: Langerhans cell
histiocytosis. Arch Dis Child. 94:904–908. 2009. View Article : Google Scholar
|
|
10
|
Histocyte Society. 2009 Langerhans cell
histiocytosis evaluation and treatment guidelines. Histocyte
Society online. 2009, cited 2010-02. Available from: http://www.histiocytesociety.org/site/c.mqISL2PIJrH/b.4442715/k.A339/Treatment_Plans.htm.
|
|
11
|
Henter JI, Tondini C and Pritchard J:
Histiocyte disorders. Crit Rev Oncol Hematol. 50:1572004.
View Article : Google Scholar : PubMed/NCBI
|
