Severe sclerosing cholangitis after Langerhans cell histiocytosis treated by liver transplantation

Tang, Yunhua; Zhang, Zhiheng; Chen, Maogen; Wang, Ju; Wang, Dongping; Ji, Fei; Ren, Qingqi; Guo, Zhiyong; He, Xiaoshun

doi:10.1097/md.0000000000005994

Cited by 15 publications

(15 citation statements)

References 10 publications

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“…Our initial search yielded 131 unique records, 44 of which were retrieved for full‐text assessment (39 from the systematic search and 5 from the snowball methodology). Ultimately, 26 nonoverlapping studies ( 10,15‐39 ) reporting on 50 patients were included in our systematic review (Table 2, Table 3, and Fig. 3).…”

Section: Resultsmentioning

confidence: 99%

Liver Transplantation for Langerhans Cell Histiocytosis: A US Population‐Based Analysis and Systematic Review of the Literature

Ziogas

Kakos²,

et al. 2021

Liver Transpl

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Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder. Liver involvement is seen in 10.1% to 19.8% of patients with LCH and can lead to secondary sclerosing cholangitis requiring liver transplantation (LT). We describe the characteristics and outcomes of patients undergoing LT for LCH. All patients undergoing a first LT for LCH in the United States were identified in the Scientific Registry of Transplant Recipients (SRTR) database (1987-2018). The Kaplan-Meier curve method and log-rank tests evaluated post-LT survival. A systematic literature review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) statement. A total of 60 LCH LT recipients were identified in the SRTR, and 55 patients (91.7%) were children with median total bilirubin levels at LT of 5.8 mg/dL (interquartile range [IQR], 2.7-12.9). A total of 49 patients (81.7%) underwent deceased donor LT (DDLT). The 1-year, 3-year, and 5-year patient survival rates were 86.6%, 82.4%, and 82.4%, respectively. The systematic review yielded 26 articles reporting on 50 patients. Of the patients, 41 were children (82.0%), 90.0% had multisystem LCH, and most patients underwent DDLT (91.9%; n = 34/37). Pre-LT chemotherapy was administered in 74.0% and steroids in 71.7% (n = 33/46) of the patients, and a recurrence of LCH to the liver was reported in 8.0% of the patients. Of the 50 patients, 11 (22.0%) died during a median follow-up of 25.2 months (IQR, 9.0-51.6), and the 1-year patient survival rate was 79.4%. LT can be considered as a feasible life-saving option for the management of liver failure secondary to LCH in well-selected patients.

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Section: Resultsmentioning

confidence: 99%

Liver Transplantation for Langerhans Cell Histiocytosis: A US Population‐Based Analysis and Systematic Review of the Literature

Ziogas

Kakos²,

et al. 2021

Liver Transpl

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“…At an early stage of disease, Langerhans cells cluster in the lobules and portal tracts, the extent of which ranges from small granulomatous foci to large tumor-like masses. Langerhans cells are commonly accompanied by a mixture of other inflammatory cells, where eosinophils frequently predominate [7][8]. In portal tract, Langerhans cells may surround and infiltrate the bile ducts, and may cause bile duct injury and proliferation [7].…”

Section: Discussionmentioning

confidence: 99%

“…Clinically patients at this stage manifest with severe cholestasis and liver failure. A subset of patients progresses to develop cirrhosis [8][9].…”

Section: Discussionmentioning

confidence: 99%

Hepatic Langerhans Cell Histiocytosis (LCH) Presenting as a Harbinger of Multisystem LCH

Ells

Arslan

et al. 2020

Cureus

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Langerhans cell histiocytosis (LCH) is a rare systemic disorder characterized by an infiltration of CD1a+/langerin+ histiocytes, commonly involving bone, skin, and lymph nodes in children. Hepatic involvement is rarely observed in multisystem LCH. We describe an exceptional case of hepatic LCH in an adult preceding the diagnosis of multisystem LCH, mimicking antimitochondrial antibody (AMA)-negative primary biliary cholangitis (PBC). A 65-year-old man presented with intermittent pruritus, weakness, dyspnea, fever, and chills that have been progressive for four years. Physical examination was unremarkable. Laboratory work revealed cholestatic biochemistry profile. Liver biopsy showed portal non-necrotizing granuloma encasing a damaged duct (florid duct lesion), and multifocal lobular Kupffer cell clusters, suggestive of PBC. Tests for autoimmune diseases including AMA were negative. Endoscopic retrograde cholangiopancreatography (ERCP) was negative for biliary obstruction. One month after the liver biopsy, he developed flaky, red, and burning rash on the right scalp, forehead, and epigastric skin. A skin biopsy at an outside institution revealed LCH. Subsequent reexamination of the liver biopsy showed that the histiocytes within the florid duct lesion were positive for CD1a and S-100. Concurrently, a small focus of LCH was noted in his gastric biopsy performed for gastritis symptoms. Hepatic LCH may mimic AMA-negative PBC histologically and clinically and may present as a harbinger of multisystem LCH. While rendering the diagnosis would be challenging without prior history of LCH and with focal involvement, awareness of such presentation and communication with clinical colleagues may be helpful.

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“…Most cases with hepatic infiltration show marked tropism of the bile ducts. Active infiltrations of the bile ducts cause mucosal injury and fibrosis that lead to a progressive pattern of SSC, ultimately resulting in biliary cirrhosis[ 11 , 15 , 16 ]. There is destruction of the walls of bile ducts which may lead to cystic dilatation with intraluminal biliary sludge and rupture (Figure 1B ).…”

Section: Liver Pathology In Lchmentioning

confidence: 99%

Current perspectives on the role of liver transplantation for Langerhans cell histiocytosis: A narrative review

Menon¹,

Rammohan²,

Vij³

et al. 2022

WJG

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Langerhans cell histiocytosis (LCH) is a malignant disease of the histiocytes involving various organ systems. The spectrum of liver involvement in LCH ranges from mild transaminitis to end-stage liver disease. The hallmark of hepatic LCH is secondary sclerosing cholangitis, which manifests due to a progressive destruction of the biliary tree by malignant histiocytes. Chemotherapy remains the mainstay of treatment for active LCH. Early recognition, diagnosis and a systematic approach to the management of LCH can ameliorate the disease process. Nonetheless, the liver involvement in these patients may progress despite the LCH being in remission. Liver transplantation (LT) remains central in the management of such patients. Various facets of the management of LCH, especially those with liver involvement remain unclear. Furthermore, aspects of LT in LCH with regards to the indication, timing and post-LT management, including immunosuppression and adjuvant therapy, remain undefined. This review summarises the current evidence and discusses the practical aspects of the role of LT in the management of LCH.

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Severe sclerosing cholangitis after Langerhans cell histiocytosis treated by liver transplantation

Cited by 15 publications

References 10 publications

Liver Transplantation for Langerhans Cell Histiocytosis: A US Population‐Based Analysis and Systematic Review of the Literature

Liver Transplantation for Langerhans Cell Histiocytosis: A US Population‐Based Analysis and Systematic Review of the Literature

Hepatic Langerhans Cell Histiocytosis (LCH) Presenting as a Harbinger of Multisystem LCH

Current perspectives on the role of liver transplantation for Langerhans cell histiocytosis: A narrative review

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