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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two children with biopsy-proven LCH underwent successful hepatic transplantation for end-stage liver disease. These patients were thought not to have active LCH disease at the time of transplantation, although one had developed a new osteolytic lesion a few months before the operation and the other had suspicious osteolytic lesions at the time of transplantation. The histologic examination of the excised liver showed features consistent with primary sclerosing cholangitis. The two patients had an excellent recovery with no evidence of progression of LCH or recurrence of the underlying disease in the hepatic allograft at 1 and 3 years after organ transplantation.
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PMID:Liver transplantation in Langerhans' cell histiocytosis (histiocytosis X). 199 20

Histiocytosis X rarely disseminates in an adult. The authors describe an unusual patients who presented with multiple areas of cutaneous and bone involvement. During the course of his disease he developed massive hepatomegaly. Aggregates of vacuolated histiocytes were found on liver biopsy. He subsequently developed diabetes mellitus complicated by ketoacidosis. Both his hepatomegaly and diabetes resolved spontaneously. No pancreatic nor pituitary abnormalities were identified. The combination of histiocytosis X, hepatomegaly, and diabetes mellitus has not been previously reported. The medical literature is reviewed with an emphasis on disseminated histiocytosis X in adults and the mechanism of glucose intolerance in liver disease.
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PMID:Reversible hepatomegaly and diabetes mellitus in an adult with disseminated histiocytosis X. 218 Feb 96

Liver biopsies were done on 20 patients with histiocytosis X (HX) as part of pretreatment evaluation prior to entry on two Childrens Cancer Study Group protocols. Seventeen patients had hepatomegaly, and seven had one or more abnormal laboratory parameters using Lahey's criteria for liver dysfunction. Nineteen of 20 specimens showed various abnormalities of the portal triads. A single biopsy revealed normal liver. Among the changes were triaditis, bile duct proliferation, variable fibrosis with histiocytic infiltrates, and cirrhosis. One patient had typical granulomas of HX within the liver parenchyma in addition to portal triaditis. Patients with larger livers and dysfunction tended to show more marked histologic abnormalities in the portal triads. However, correlations among liver size, function, and pathology showed considerable overlap. Early death among these patients was more likely to be associated with progressive HX in other sites and/or infection. Death from cirrhosis and liver failure per se occurred in one patient 4 years after initial biopsy, but five other children had evidence of cirrhosis on biopsy or at autopsy. The majority of patients with triaditis initially did not have clinical evidence of progressive liver disease although four expired with other manifestations of HX or infection. Conversely, patients showing fibrohistiocytic changes or cirrhosis initially were likely to have continuing or progressive liver disease. Although the liver histology was not diagnostic of HX, the types of portal changes usually predicted the subsequent course of liver disease.
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PMID:Pretreatment liver biopsy in 20 children with histiocytosis X: a clinicopathologic correlation. 240 53

We cared for 124 pediatric patients with a histologic diagnosis of Langerhans' cell histiocytosis (histiocytosis X) over a period of 14 years. Clinical, laboratory, and radiographic findings were analyzed. The most frequent manifestations were bone lesions, lymph node involvement, and skin infiltration. Liver disease was noted in 50% of patients and lung disease in 23%; hematologic changes were also frequent. Dysfunction and involvement of these three organ systems, plus age of onset, distinguished the group of patients with the highest mortality. All patients with generalized disease or organ dysfunction were treated with systemic chemotherapy. The actuarial survival curve at 10 years was 63%.
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PMID:Langerhans cell histiocytosis: clinical experience with 124 patients. 326 10

Langerhans' cell histiocytosis (LCH) represents 15% to 20% of sclerosing cholangitis (SC) in children. In LCH-associated SC, a very poor response to chemotherapy has been reported, and spontaneous prognosis is very bad. Few cases of orthotopic liver transplantation (OLT) for LCH have been reported and little is known about the risk of recurrence and the effect of immunosuppression after OLT. Since 1986, five children (mean age +/- SD, 12.6 +/- 3.6 years) underwent OLT for SC complicating LCH. All patients presented with growth retardation, and severe liver disease including repeated episodes of variceal bleeding (n = 5), liver insufficiency (n = 4), jaundice (n = 5), and ascitis (n = 4). Four patients presented with previous abdominal surgery (mesocaval shunt in two and explorative laparotomy in two). OLT was performed using a whole (n = 2) or a reduced liver graft (n = 3). Pathological examination of the recipient liver showed biliary cirrhosis in all cases. Three patients are alive and well 9 to 88 months after OLT. None presented with recurrence of LCH or SC within the liver graft or in other organs. General condition (including growth and puberty) improved dramatically in the three surviving patients. Absence of recurrence after up to 7 years of follow-up after liver transplantation in our patients, as well as in all other reported cases, is encouraging and shows that OLT may be indicated in end-stage liver disease complicating LCH. Furthermore, cyclosporine may be beneficial for preventing recurrence of LCH, in which autoimmunologic mechanisms have been advocated.
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PMID:Pediatric liver transplantation for Langerhans' cell histiocytosis. 780 45

Langerhans' cell histiocytosis (LCH) is a rare disorder of unknown etiology and pathogenesis. End-stage chronic liver disease is one presentation and orthotopic liver transplantation (OLT) has been reported in 17 cases, with variable resolution of LCH lesions postoperatively. We report a case of multisystem LCH with end-stage liver disease treated by OLT and review the overall results of OLT for children with LCH.
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PMID:Liver transplantation for Langerhans' cell histiocytosis--a case report and literature review. 890 Mar 19

Langerhans' cell histiocytosis (LCH) of the liver is uncommon. When seen, it is part of multifocal disease and can present as biliary obstruction. We present a case of sclerosing biliary disease with a solitary LCH lesion and no evidence of systemic disease. We postulate that the LCH is a secondary phenomenon, arising against a background of a complex, familial liver disease. This case also raises the possibility that some instances of idiopathic sclerosing cholangitis may follow cryptic LCH of the bile ducts.
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PMID:Langerhans' cell granuloma confined to the bile duct. 918 24

Three cases of Langerhans' cell histiocytosis with unusual clinical and histopathologic features are described. The first two cases illustrate diagnostic pitfalls that underscore the importance of considering Langerhans' cell histiocytosis in the differential diagnosis of purpuric papular eruptions of the scalp and intertriginous areas, particularly in association with hypothalamic, pituitary, or liver disease. The third case is the first report of Langerhans' cell histiocytosis presenting as a vesicular eruption.
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PMID:Langerhans' cell histiocytosis in adults. 927 May 36

Neonatal lupus erythematosus (NLE) is an autoimmune disease characterized primarily by transient skin lesions and/or permanent congenital heart block. Other clinical findings include self-limited cytopenias and liver disease. The syndrome results from the passive transfer of maternal anti-SSA, anti-SSB, or anti-U1RNP autoantibodies to the fetus across the placenta. The cutaneous manifestations are generally analogous to those of subacute cutaneous lupus erythematosus (SCLE) and consist of small, erythematous macules that progress to annular plaques with delicate scaling. The skin lesions usually resolve within the first 6 months of life as maternal autoantibodies are cleared from the infant's circulation. We describe a patient with cutaneous NLE with hepatic and hematologic manifestations. The clinical presentation was atypical, with splenomegaly and petechiae at birth followed by a crusting, papulosquamous skin eruption of the scalp and face mimicking Langerhans cell histiocytosis (LCH).
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PMID:Neonatal lupus erythematosus mimicking langerhans cell histiocytosis. 1265 18

We report on the difficult differential diagnosis of liver involvement in disseminated Langerhans' cell histiocytosis (LCH). Three years after treatment of LCH involving the skull and pelvic bones, an 18-year-old girl presented with abdominal pain and cholestatic liver disease. At this time, liver biopsy showed portal infiltrates which were diagnosed as chronic non-suppurative destructive cholangitis. Two years later, she was icteric under progredient hepatic failure. A second liver biopsy revealed biliary fibrosis and granulomatous inflammation with destruction of the portal bile ducts. The morphological changes in both liver biopsies could be identified as LCH by immunohistochemical detection of CD1a and S-100-positive Langerhans' cells. Morphological changes and clinical findings in LCH of the liver may resemble primary sclerosing cholangitis or chronic non-suppurative destructive cholangitis. Therefore, LCH is an important differential diagnosis of chronic destructive cholangitis with cholestatic liver disease, especially in children and young adults. The diagnosis can be verified by S-100 and CD1a immunohistochemistry.
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PMID:[Langerhans' cell histiocytosis of the liver. Differential diagnosis of a rare chronic destructive sclerosing cholangitis]. 1267 1


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