Liver transplantation in an adult with sclerosing cholangitis due to Langerhans cell histiocytosis

Griffiths, William; Davies, Susan; Gibbs, Paul; Thillainayagam, Andrew V.; Alexander, Graeme

doi:10.1016/j.jhep.2005.12.024

Cited by 25 publications

(15 citation statements)

References 8 publications

(12 reference statements)

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“…[6] At present, there are only 6 cases of adults with LCH underwent LT that have been published. [4,7,8] We reported a patient with severe SC and was ultimately treated with LT and acquired excellent prognosis. Due to limited experience in rare disease, LT would be an effective therapy for severe SC after LCH in adults, which help us get a better understanding of multisystem LCH and improved diagnosis and treatment.…”

Section: Discussionmentioning

confidence: 99%

Severe sclerosing cholangitis after Langerhans cell histiocytosis treated by liver transplantation

et al. 2017

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Background:Langerhans cell histiocytosis (LCH) is a rare hematopoietic disorder of unknown pathogenesis. LCH diseases may occur in a single organ or multisystem organ. The patients with multisystem involvement usually have a poor prognosis. Liver involvement in multisystem LCH results in severe complications, such as obvious sclerosing cholangitis (SC) with jaundice.Methods:We reported a 31-year-old man developed severe SC due to multisystem LCH and was successfully treated by liver transplantation (LT). In addition, we firstly used tacrolimus and mycofenolate mofetil as immunosuppressants to treat LCH after LT.Results:We performed the immunosuppressants to deal with the LCH after LT, now the patient is currently well with normal liver function and no evidence of recurrence of LCH for 4 and a half years follow-up.Conclusion:LT should be recommended as an effective treatment for these adults with severe SC due to multisystem LCH. Finally, using tacrolimus and mycofenolate mofetil as immunosuppressants to treat LCH might be favorable to prevent LCH recurrence.

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Section: Discussionmentioning

confidence: 99%

Severe sclerosing cholangitis after Langerhans cell histiocytosis treated by liver transplantation

et al. 2017

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“…724,725 In particular, patients with hepatic involvement have a worse prognosis with survival at 3 years of 96.7% for those without, but only 51.8% for those with liver involvement. 726 In patients with cirrhosis and endstage liver failure secondary to LCH sclerosing cholangitis, orthotopic liver transplantation has been proven a successful therapeutic option 713,727,728 with 67% of patients living long-term after surgery (median follow-up 5.8 years, range 2.1-7.5 years). A significant number of LCH patients have developed post-transplant lymphoproliferative disorders, which seems related to a higher incidence of refractory rejection.…”

Section: Kawasaki Diseasementioning

confidence: 99%

Developmental abnormalities and liver disease in childhood

Portmann¹,

Roberts²

2012

MacSween's Pathology of the Liver

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“…In adults, sclerosing cholangitis, as a late complication in Langerhans cell histiocytosis, has only been described in a few cases associated with poor prognosis, high mortality and LT as the only therapeutic option. (121,(124)(125)(126)(127)(128)(129)(130).…”

Section: Causes Of Secondary Sclerosing Cholangitismentioning

confidence: 99%

Evolving concepts in primary sclerosing cholangitis

Krones

Graziadei

Trauner

et al. 2011

Liver International

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Keywords autoimmune sclerosing cholangitis -children -IgG4-associated sclerosing cholangitisinflammatory bowel diseases -primary sclerosing cholangitis -sclerosing cholangitis in critically ill patients -secondary sclerosing cholangitis AbstractPatients suffering from primary sclerosing cholangitis (PSC) show considerable differences regarding clinical manifestations (i.e. large duct versus small-duct PSC, presence or absence of concomitant inflammatory bowel disease), disease progression, risk for malignancy and response to therapy, raising the question whether PSC may represent a mixed bag of diseases of different aetiologies. The growing list of secondary causes and diseases 'mimicking' or even overlapping with PSC (e.g. IgG4-associated sclerosing cholangitis), which frequently causes problems in clear-cut discrimination from classic PSC and the emerging knowledge about potential disease modifier genes (e.g. variants of CFTR, TGR5 and MDR3) support such a conceptual view. In addition, PSC in children differs significantly from PSC in adults in several aspects resulting in distinct therapeutic concepts. From a clinical perspective, appropriate categorization and careful differential diagnosis are essential for the management of concerned patients. Therefore, the aim of the current review is to summarize current and evolving pathophysiological concepts and to provide up-to-date perspectives including future treatment strategies for PSC.

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Liver transplantation in an adult with sclerosing cholangitis due to Langerhans cell histiocytosis

Cited by 25 publications

References 8 publications

Severe sclerosing cholangitis after Langerhans cell histiocytosis treated by liver transplantation

Severe sclerosing cholangitis after Langerhans cell histiocytosis treated by liver transplantation

Developmental abnormalities and liver disease in childhood

Evolving concepts in primary sclerosing cholangitis

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