Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Diabetes insipidus is a clinical syndrome characterized by the excretion of copious volumes of dilute urine combined with persistent intake of abnormally large quantities of fluid. There are two general forms of the disease, central (vasopressin deficient) and nephrogenic (vasopressin resistant). Diabetes insipidus of central origin most often results from lesions in the hypothalamic-neurohypophyseal axis. Twenty-six cases of central diabetes insipidus were evaluated with the use of high-field-strength MR imaging. A wide variety of precipitating conditions were found, including Langerhans cell histiocytosis, neoplasia, trauma, and infection. A thickened pituitary infundibulum was seen in most patients, and an absence of high intensity signal in the posterior pituitary lobe on T1-weighted images was seen in every case. Analysis of stalk morphology; associated brain findings; and correlation with the patient's age, sex, history, and radiographs of other body parts improved diagnostic specificity. When combined with clinical information, MR imaging is able to provide a specific diagnosis in almost all cases of central diabetes insipidus.
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PMID:MR imaging of the brain in patients with diabetes insipidus. 827 42

An unusual case of cutaneous and hypothalamic histiocytosis X (HX) is reported. The hypothalamic involvement occurred as a tumor that mimicked a chiasm glioma on computed tomography angioscanning. Magnetic resonance imaging after gadolinium injection localized the tumor within the third ventricle floor. The HX origin of the tumor was confirmed by histological examination of hypothalamic biopsies obtained by transventricular endoscopy. The results of endocrine evaluation were consistent with anterior panhypopituitarism resulting from a multiple releasing-hormone secretory defect, but there was no diabetes insipidus. This unusual endocrine aspect has not been previously described in the field of hypothalamic HX. Lastly, the tumor was insensitive to low dose megavoltage radiation therapy. This unusual case stresses the superiority of magnetic resonance imaging over computed tomography scanning in the assessment of suprasellar tumors and emphasizes the usefulness of transventricular endoscopy in these cases.
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PMID:Histiocytosis X of the hypothalamus. 206 69

The histiocytoses, whether reactive or neoplastic, can be related histologically and immunophenotypically to their normal counterparts within the histiocytic system. This system has two subsets: The dendritic (antigen-presenting) cells and the phagocytic histiocytic (antigen-processing) cells. Dermatopathic lymphadenitis and Langerhans cell histiocytosis (histiocytosis X) are reactive proliferative disorders of dendritic cells. Malignancies of dendritic cells exist, but they are very rare. Benign proliferations of phagocytic histiocytes include the hemophagocytic syndromes, both familial and reactive, as well as sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and histiocytic necrotizing lymphadenitis (Kikuchi's disease). Neoplasms of phagocytic histiocytes include acute monocytic leukemia and the very rare entities, malignant histiocytosis and true histiocytic lymphoma. The latter must be distinguished from sinusoidal, large cell anaplastic lymphomas.
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PMID:The histiocytoses: clinical presentation and differential diagnosis. 215 Mar 25

A case of localized histiocytosis X of the penis was reported. The patient was a 9-year-old boy who had several tumor lesions on the glans penis and inner layer of prepuce. Biopsy specimens were studied by H-E staining and immunohistochemical staining for S-100 protein (S-100), lysozyme (Lys), leucocyte common antigen (LCA), and Leu-M1. They revealed diffuse infiltration of many atypical histiocytes, which were shown to be S-100+, Lys+, LCA- and Leu-M1-. This indicates that these cells were derived from T-zone histiocyte system. After complete remission of these tumors, the other one arose from anal mucosa. In the literature we could find only one case of a primary penile lesion reported by Myers and others.
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PMID:[A case of histiocytosis X of the penis]. 229 25

Using a modified method of Con A, LCH or PHA-E affinity crossed-line immunoelectrophoresis, we studied AFP subfractions in 78 sera including 58 from patients with primary hepatoma, 11 from patients with hepatic metastasis of gastric cancer and 9 from patients with germ cell tumors of the gonads (yolk sac tumor, immature solid teratoma or mature solid teratoma). It was found that AFP in primary hepatoma, metastatic hepatoma or germ cell tumors of the gonads were differently glycosylated, and different patterns of AFP subfractions identified by Con A, LCH or PHA-E affinity crossed-line immunoelectrophoresis facilitated a differential diagnosis of such AFP related malignancies.
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PMID:[Serum AFP subfractions in patients with hepatic cancer or germ cell tumor of the gonads]. 241 63

Using a modified method of concanavalin A (Con A), lentil lectin (LCH) or phytohemagglutinin-E (PHA-E) affinity crossed-line immunoelectrophoresis, alpha-fetoprotein (AFP) subfractions were studied in 33 samples of human amniotic fluid obtained between 41 and 287 days of gestation. Fetal tissues (yolk sac, liver, stomach and small intestine) obtained from a fetus of 68 days' gestation were incubated for 24 hours and AFP subfractions in the culture fluid examined. AFP in control amniotic fluids yielded two subfractions (types a and b) with Con A, three subfractions (types A, B and C) with LCH, and four subfractions (types W, X, Y and Z) with PHA-E. Serial changes of AFP subfractions in the amniotic fluid, as well as in the incubation study, indicated that the yolk sac and the gastrointestinal tract were responsible for the production of the Con A non-reactive subfraction (type b), the LCH weakly-reactive subfraction (type B) and the PHA-E reactive subfraction (types W and X), at an early stage of gestation. The Con A reactive subfraction (type a), LCH reactive subfraction (type A), PHA-E weakly-reactive subfraction (type Y) and PHA-E non-reactive subfraction (type Z) were assumed to be produced mainly by yolk sac, liver or gastrointestinal tract. 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. 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.
Tumour Biol 1985
PMID:Alpha-fetoprotein subfractions in amniotic fluid identified by a modification of the method of concanavalin A, lentil lectin or phytohemagglutinin-E affinity crossed-line immunoelectrophoresis. 241 31

S-100 protein has been used as a marker of various lesions, including peripheral nerve sheath, cartilaginous and salivary gland tumors, chordomas, histiocytosis X, and melanomas, among others. The list of neoplasms that can express S-100 protein continues to expand. It has been suggested that staining for S-100 protein may be of aid in the differential diagnosis of amelanotic melanoma versus poorly differentiated tumors. Three hundred fifty primary and metastatic adenocarcinomas from various sites were immunostained for S-100 protein with the use of a commercially available polyclonal antibody. Forty-two percent of the adenocarcinomas tested expressed S-100 protein to varying degrees. The relative incidence of S-100-positive tumors varied with the primary sites, some expressing S-100 protein more often than others. A primary neoplasm able to express S-100 protein was usually associated with metastatic foci also expressing this marker. However, occasionally, a primary S-100-positive tumor was associated with metastasis that lacked expression of S-100. This study emphasizes the importance of testing for a panel of tumor markers in the evaluation of poorly differentiated tumors and cautions on possible difficulties that may arise in the interpretation of immunocytochemistry results.
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PMID:S-100 protein expression by primary and metastatic adenocarcinomas. 244 69

FITC-Con A staining as a rapid diagnostic method for tumor cells was applied to the tumors smeared on glass slide and section specimens to evaluate the reactivity with FITC-Con A. Good staining results were obtained in smear specimens with clear fluorescence on the membrane of tumor cells. Con A and LCH lectins bound well with tumor cells to produce strong fluorescence in comparison with PEA and DBA. It indicates that tumor cells expressed dominantly the receptors of alpha-D-glucose and alpha-D-mannose sugar chain on the membrane of tumor cells. From these results it was concluded that FITC-Con A staining method applied to smear specimens is more advantageous in the rapidity and the simplicity for tumor cell diagnosis than section specimen method.
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PMID:[Rapid diagnosis of experimental tumor by FITC-Con A lectin--a comparative study of smear and section specimens]. 245 71

In this study fresh frozen tissue samples of benign osseous tumors (five non-osteogenic fibromas, one fibrous dysplasia, one chondromyxoidfibroma), tumors of uncertain biological behaviour (eight cases of histiocytosis X, two giant-cell tumors), and of malignant intraosseous tumors (two malignant fibrous histiocytomas, two malignant histiocytosis, four osteosarcomas, one chondrosarcoma and two Ewing sarcomas) were studied with a panel of monoclonal antibodies reactive with monocyte/macrophages and various types of dendritic cells. In addition, tumors were further defined with a broad spectrum of antibodies against filamentous proteins and lymphocyte differentiation antigens. The specimens were stained with a triple-layer immunoalkaline phosphatase protocol. Tumors stained with these antibodies could be roughly divided into two groups. The first group comprised tumors with one predominant cell population reactive with one particular monoclonal antibody. In this group, cases of histiocytosis X were found to be consistently labelled with CD-1 antibodies. The giant-cell tumors showed a very homogeneous staining with certain monocyte/macrophage antibodies (Ki-M8). Nevertheless, even in these tumors, heterogeneity was demonstrated by the occurrence of cells with monocytic differentiation in histiocytosis X and conversely by the occurrence of cells with differentiation antigens of the dendritic cell system in giant-cell tumors. An exception has to be made for the two cases of malignant histiocytosis examined. These tumors were selectively labelled with antibodies against monocyte/macrophages (Ki-M8, IOM-1). The second group comprised tumors showing a high degree of heterogeneity demonstrated by the varying amounts of tumor cells reacting with the applied markers of the monocyte/macrophage and dendritic cell systems. In most cases it was difficult to ascribe labelled cells to the tumor cell population as opposed to an "innocent bystander" inflammatory cell population. This distinction was especially difficult in malignant fibrous histiocytomas underlining the current concept that these tumors are of primitive mesenchymal rather than true histiocytic origin.
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PMID:Histiocytic differentiation in benign and malignant bone tumors. 246 63

Profiles of concanavalin A (Con A) and lentil agglutinin (LCH) affinity immunoelectrophoresis were compared for serum alpha-fetoprotein (AFP) from patients with yolk sac tumors and carcinomas of the gastrointestinal tract, in order to find some correlations between peaks of AFP subfractions detectable by two different lectins, and to investigate whether or not it is possible to prove that the binding of AFP to LCH is weakened to some extent if a fucosylated sugar chain has, in addition, a bisect N-acetylglucosamine (GlcNAc) attached to the beta-linked mannose. The results obtained with our improved techniques tend to indicate that a Con A-reactive AFP subfraction (peak a) corresponds to an LCH strongly reactive AFP (peak A), while a Con A-nonreactive AFP (peak b) corresponds to an LCH weakly reactive AFP (peak B). the authors consider the present data sufficient to support the above explanation.
Tumour Biol 1989
PMID:Analysis of lectin affinity immunoelectrophoretic profiles of serum alpha-fetoprotein from patients with yolk sac tumors and carcinomas of the gastrointestinal tract: correlations with molecular structures. 248 Jun 30


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