Disease relevance of LCH

• Because Langerhans cells (LC) in peripheral tissues are generally "immature" cells with poor lymphostimulatory activity, the contribution of immune responses initiated by LC to the pathogenesis of pulmonary LC histiocytosis (LCH) has been uncertain [1].
• In this study we demonstrate that LC accumulating in LCH granulomas are phenotypically similar to mature lymphostimulatory dendritic cells present in lymphoid organs [1].
• Because these patients are susceptible to developing severe respiratory insufficiency, they should also be considered for treatment as soon as an effective therapy for LCH is available [2].
• Two children with biopsy-proven LCH underwent successful hepatic transplantation for end-stage liver disease [3].
• BACKGROUND: Pulmonary involvement with Langerhans cell histiocytosis (LCH, formerly known as histiocytosis-X) presents as an interstitial process in children and adults either with or without symptoms [4].

Psychiatry related information on LCH

• The etiology of LCH remains unknown, although the evidence indicates that it is a clonal proliferative disorder of Langerhans cells, it has also been characterized as reactive disorder, neoplastic process and a berrant immune response [5].
• Among those trying to control weight, a significantly higher proportion of current LCHP diet users reported eating fewer calories and meeting physical activity recommendations (38.0%) compared with nonusers (29.8%) [6].

High impact information on LCH

• Finally, LCH granulomas were the only site where CD154-positive T cells could be identified in close contact with LC intensely expressing CD40 Ags [1].
• Taken together, these results strongly support the idea that an abnormal immune response initiated by LC may participate in the pathogenesis of pulmonary LCH, and suggest that therapeutic strategies aimed at modifying the lymphostimulatory phenotype of LC may be useful in the treatment of this disorder [1].
• For R = tBu, the ionic carbenium thiocyanates [LCH]+ SCN- dominate the product spectrum and the respective thioureas are obtained in low yield [7].
• The left kidney of a rat was perfused either with 200 micrograms of Lens culinaris hemagglutinin (LCH) plus 1 mg of 6D1 (IgG1 fraction) (Group I and III) or with LCH only (Group II) through a cannula placed in the left renal artery [8].
• The first major stride toward understanding LCH was taken when ultrastructural studies identified the proliferating cells as part of the Langerhans (dendritic) cell system [9].

Chemical compound and disease context of LCH

• Etoposide should be seriously considered as therapy for patients with LCH in whom the toxicity/benefit ratio of steroid therapy is unacceptably high [10].

Biological context of LCH

• The present study explores a class of nonpolar binding sites reportedly present adjacent to the carbohydrate binding site in PSA, LCH, and VFA [11].
• BACKGROUND: Defective male sex differentiation in patients with hypoplasia of Leydig cells (LCH) is caused by deficient LH receptor signal transduction [12].
• We also found that the LCH non-reactive subfraction (type C) was synthesized either by liver or by the gastrointestinal tract at an early stage of gestation [13].
• Testicular histology and hormonal profile of the patient is typical of LCH [14].
• We found that type a of Con A or type C of LCH was the only subfraction present in maternal circulation at the second or third trimester [15].

Anatomical context of LCH

• Moreover, the agreement concerning diagnostic criteria provides a solid foundation for current clinical trials and for laboratory research regarding the possible roles of the immune system, clonality, and cytokines in the etiology of LCH [9].
• Descriptions of patients with LCH whose disease is confined to lymph nodes are rare [16].
• At term, type a of Con A, type C of LCH and types Y and Z of PHA-E were the main subfractions in amniotic fluid, assumed to be produced by the fetal liver [13].
• With combined use of concanavalin A (Con A) and lentil agglutinin (LCH) binding tests, AFPs were classified into three subtypes: benign hepatic condition type (six patients), hepatocellular carcinoma type (nine patients) and yolk sac type (12 patients) [17].
• The mean initial leukocyte count was higher than in the non-HLH/LCH group (270,700 +/- 60,677 microl(-1) vs 134,141 +/- 5,663 microl(-1); p = 0.074) [18].

Associations of LCH with chemical compounds

• Concanavalin A (Con A) and agglutinins from the pea (PSA), lentil (LCH), and fava bean (VFA) constitute a group of D-mannose/D-glucose binding legume lectins [11].
• The hapten inhibition assay results reveal that the presence of a methyl or methylene group at the O-2 or O-3 position of the sugar is essential for hydrophobic interaction with PSA, LCH, and VFA [11].
• Etoposide may become a "first-line" drug in the treatment of systemic LCH, especially when the side effects of steroid therapy are considered unacceptable [19].
• Comparison of the hydrolysis of polyethylene terephthalate fibers by a hydrolase from Fusarium oxysporum LCH I and Fusarium solani f. sp. pisi [20].
• The hydrolase from a newly isolated Fusarium oxysporum strain (LCH 1) was more efficient in releasing terephthalic acid from PET fibers compared to the enzyme from F. solani f. sp. pisi DSM 62420 when equal amounts of p-nitrophenyl butyrate-hydrolyzing activity were employed [20].

Regulatory relationships of LCH

• We conclude that Langerin is coexpressed with CD1a on LCs and LCH [21].

Other interactions of LCH

• Several cells in LCH florid lesions showed immunoreactivity for both IL-1 alpha and beta [22].
• Fractionation of AFP was carried out by concanavalin A (Con A) or lentil lectin (LCH) crossed-line affinity immunoelectrophoresis [23].
• Clinical and epidemiologic studies of familial hemophagocytic lymphohistiocytosis in Japan. Japan LCH Study Group [24].
• Such treated microfilariae showed a weak binding of Concanavalin A (Con A), and lectins of lentil (LCH) and of Limulus polyphemus (LPA) [25].
• All 12 non-LCH histiocytic disorders are negative for Langerin in the histiocytes of interest [21].

Analytical, diagnostic and therapeutic context of LCH

• 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 [3].
• 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 [3].
• Clinically, different patterns of AFP subfraction identified by Con A or LCH crossed-line affinity immunoelectrophoresis facilitate a differential diagnosis of primary hepatoma and hepatic metastasis of gastric cancer, in cases of elevated serum AFP levels [23].
• CONCLUSIONS: A multicenter study of therapy for pulmonary LCH is the obvious answer to this dilemma [4].
• CASE: We present a case of LCH confined to lymph nodes initially diagnosed by fine needle aspiration (FNA) cytology in a 43-year-old male [16].

References