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)

The labeling efficiency, biological activity and biodistribution of 125I labeled and 67Ga chelating agent conjugated lectins were investigated. Pisum sativum agglutinin (PSA) and Lens culinaris agglutinin (LCH) were efficiently labeled with 67Ga using bifunctional chelating agents such as diethylenetriaminepentaacetic acid (DTPA) and deferoxamine (DFO), whereas labeling with 125I was significantly less efficient. The agglutinating activity of these lectins towards Ehrlich ascites tumor (EAT) cells was retained on conjugation with DFO, but not with DTPA. The in vitro binding ratio of 67Ga-DFO-lectins for EAT cells was almost the same as that of 125I-lectins. However, the value was significantly decreased in the case of 67Ga-DTPA-lectins. In the biodistribution study of radiolabeled lectins in Ehrlich solid tumor (EST) bearing mice, the accumulation of radioactivity in tumor tissue was very much less with 67Ga-DTPA-lectins than with 125I-lectins. However, the concentration was significantly elevated in the case of 67Ga-DFO-lectins. While, these lectins accumulated in liver, spleen, lung, and kidney to a greater extent than 67Ga citrate, the tumor to organ ratios became very low. These low tumor to organ ratios, in contrast to 67Ga citrate, will certainly inhibit the tumor delineation, and therefore it seems that in spite of a high accumulation ratio of 67Ga-DFO-lectins in tumor tissue, these agents are not useful in tumor detection.
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PMID:Comparisons of labeling efficiency, biological activity and biodistribution among 125I-, 67Ga-DTPA-and 67Ga-DFO-lectins. 342 86

Involvement of the central nervous system by histiocytosis X is usually restricted to the parasellar region. A rare case of histiocytosis X involving the cervical meninges in a 12-month-old boy is demonstrated and the magnetic resonance features of this tumor are described.
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PMID:Cervical meningeal histiocytosis demonstrated by magnetic resonance imaging. 349 74

A case of isolated sphenoidal tumor localization in a child without any endocrinological disorder and revealing an histiocytosis X is reported. This mode of presentation is extremely rare. The diagnosis of histiocytosis X must be evoked and proved in lesions of the sellar region in children.
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PMID:[Isolated sphenoidal tumor localization in a child, disclosing histiocytosis X]. 349 62

We reviewed the Tumor Registry for 1981 at the Children's Hospital of Philadelphia to identify all the children with newly diagnosed cancer who were seen initially in the emergency department (ED). Of the 220 new patients listed, 16 (7.3%) sought initial care in the ED (1 per 4,500 ED visits). Seven had leukemia, five had non-CNS solid tumors (2 lymphoreticular, 1 Wilms', 1 neuroblastoma, and 1 ovarian), and four had CNS tumors. Among the children with leukemia, pallor (6) and decreased activity (4) were the most common complaints. Duration of symptoms ranged from 4 days to 3 weeks. Physical examination showed pallor (5), splenomegaly (4), fever (3), hepatomegaly (3), lymphadenopathy (3), and ecchymoses or petechiae (2). The complete blood count and peripheral smears were all abnormal. The five patients with non-CNS solid tumors had symptoms related to the location of their neoplasms. The patients with Wilms' tumor, neuroblastoma, and ovarian dysgerminoma had abdominal masses; the patient with lymphoma had a large, painful inguinal node; and the patient with histiocytosis X had an infiltrative rash, gingivitis, and pneumonitis. Of the four children with CNS tumors, three had headache, and one had an incidentally detected scotoma following head trauma. All four eventually had abnormal neurologic exams and computer tomographic scans, but two were discharged initially with psychiatric diagnoses. We conclude that cancer, although rare in children, occurs with greater relative frequency in the referral hospital ED than that predicted by published cancer rates from the referring hospital's ED.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Detection of cancer in the pediatric emergency department. 384 22

The authors describe a 63-year-old woman who developed a histologically distinctive malignant cutaneous neoplasm composed of large pleomorphic cells with abundant cytoplasm and multilobate, often clefted nuclei that occasionally contained small nucleoli. This neoplastic cell population metastasized to a regional lymph node already involved by a B-cell derived chronic lymphocytic leukemia expressing surface IgMk, BA-1, and OKT1. The large metastatic tumor cells lacked surface immunoglobulin, B-lymphocyte associated antigen BA-1, T-lymphocyte associated antigens OKT1 and OKT3, and the monocyte/macrophage markers lysozyme and alpha 1-antichymotrypsin. These tumor cells expressed HLA-DR antigens, adenosine triphosphatase (ATPase), OKT6, and contained S-100 protein, i.e., they expressed the phenotype peculiar to epidermal Langerhans cells. The typical clinical and histologic features of Histiocytosis X were absent. Thus, this case appears to represent a distinctive cutaneous neoplasm composed entirely of malignant cells of dendritic cell origin which, by immunophenotypic and histochemical analysis, appear to be related to epidermal Langerhans cells.
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PMID:A distinctive cutaneous malignant neoplasm expressing the Langerhans cell phenotype. Synchronous occurrence with B-chronic lymphocytic leukemia. 388 25

Chemotaxis is a property common to all free cells or unicellular microorganisms. It is not a simple spontaneous cellular migration but one which is directed towards the source or nucleus, producer of the chemotactic substance. One of the first phenomenon which is established as a defense mechanism of the organism is the attraction of polymorphonuclears. In 1955 Rebuck and Crowley described a method, "skin window" for the study of in vivo leukocyte chemotaxis. The aim of this work was to go deeper into the study of this test and to establish its clinical use. Two hundred and seventy patients from both sexes were studied and divided into five groups: Group I - 60 healthy subjects as control. Group II - 60 patients with pathologic leukocyte response: 10 cirrhotics, 15 Hodgkin's disease, 15 chronic renal insufficiency, 2 drepanocytosis and 3 sarcoidosis. Group III - 60 patients with no theoretical alterations in the leukocyte chemotaxis: 22 bronchial asthma, 23 nonlymphoid neoplasm, 13 iritis and 2 histiocytosis X. Group IV - 40 active tuberculosis patients. Group V - 30 patients with bacterial pneumonia non-tuberculosis. The Rebuck test was carried out on all patients. As lymphocyte markers, E rosettes, superficial immunoglobulins and the lymphoblast transformation test against PHA were performed on all the groups of patients. As to the results obtained, the positive responses for Groups I, II, III, IV and V were 87%, 28%, 83.3%, 45% and 63.3%, respectively. These results were evaluated in relation to the Mantoux reaction. The modified Rebuck test is useful for leukocyte chemotactic study. This was found to be altered in 13% of the healthy population.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Behavior of leukocyte chemotaxis in various clinico-immunological situations]. 389 89

A 71-year-old white man developed an increasing number of 1-to-10 mm, erythematous nodules, many with central ulceration, most prominent on the head and trunk. Biopsy of a nodule showed infiltration of the dermis and epidermis by large cells with multilobulated nuclei and numerous mitoses. Electron microscopy showed that most tumor cells contained Langerhans' cell granules. Immunohistochemical studies demonstrated a pattern of antigen expression similar to that of Langerhans' cells including Ia and Leu-6 (T6) antigens. Chest x-ray showed diffuse pulmonary infiltration and similar tumor cells were present in the sputum and urine. He developed increasing dyspnea and jaundice despite chemotherapy, and died 6 months after the onset of the disease. Autopsy showed massive tumor infiltration of the lungs, liver, spleen, and lymph nodes, and focal involvement of the myocardium, skin and bladder. Clinical and cytologic features indicated this case to be a rare example of highly malignant histiocytosis X in an elderly man.
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PMID:Malignant histiocytosis X. Report of a rapidly fatal case in an elderly man. 620 93

A rare retroperitoneal malignant xanthogranuloma is described. The patient developed malignant bone lesions followed by brain localization, and died three years later. At autopsy a very large cystic retroperitoneal tumor with osteolytic lesion of sacrum was found. A review of cases diagnosed as retroperitoneal xanthogranuloma indicates that this lesion seems to be a particular form of the wide group of malignant fibrous histiocytoma with xanthomatous and inflammatory cells. Moreover it could be a primary malignant fibrous histiocytoma of bone. Furthermore, difficulties of differential diagnosis of these tumors are discussed mainly with histiocytosis X.
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PMID:[Retroperitoneal malignant xanthogranuloma (author's transl)]. 627 95

The presence of S-100 protein was immunohistochemically studied in many types of formalin-fixed and paraffin-embedded tumors (260 cases). Peripheral nerve tumors, i.e., schwannomas, neurofibromas, granular cell tumors, and neurogenic sarcomas were demonstrated to contain variable amounts of S-100 protein in the tumor cell cytoplasm and nuclei. In ganglioneuromas and ganglioneuroblastomas, neoplastic Schwann cells or satellite cells were positive for S-100 protein. About one-half of the cases of carcinoid tumors stained weakly for S-100 protein. In addition to these nervous tissue and carcinoid tumors, chondrosarcoma, chordomas, pleomorphic adenomas of the salivary gland, and Langerhans cell granulomatosis were also shown to produce S-100 protein. In many types of breast tumors and other lesions, S-100 protein positive cells were likely to correspond to the distribution of myoepithelial cells. These results indicate that S-100 protein is not strictly specific to nervous tissue and its tumors; however, the immunohistochemical demonstration of S-100 protein can be a useful diagnostic tool in tumor diagnosis.
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PMID:An immunoperoxidase study of S-100 protein distribution in normal and neoplastic tissues. 630 Dec 96

Two hundred and two benign and malignant soft tissue lesions were studied for the presence of S-100 protein by means of the peroxidase-antiperoxidase technique on formalin-fixed, paraffin-embedded tissue. Virtually all benign nerve sheath tumors (neurofibroma, neurilemoma, and granular cell tumor) contained numerous immunoreactive S-100-positive cells. Only one-half (18 of 36) of malignant schwannomas contained the protein, suggesting that its presence is an expression of differentiation in Schwann cell tumors. S-100 protein was not identified within pure neuroblastic tumors (neuroblastoma, neuroepithelioma) but could be identified within rare cells of the ganglioneuroblastoma and within the Schwann cell component of ganglioneuroma. It was also identified within most melanocytic tumors (cellular blue nevus, clear cell sarcoma, and melanoma). In fact, its constant presence in melanoma indicates that it may prove to be an independently reliable method for diagnosing amelanotic forms. It is also sporadically present within a variety of mesenchymal lesions including lipoma, liposarcoma, synovial chondromatosis, chondrosarcoma, fibromatosis, histiocytosis X, and chordoma. Although S-100 protein is highly characteristic of neural crest-derived tumors, it is not restricted to them and, consequently, must be interpreted cautiously. It may prove helpful in select situations such as the distinction of (a) benign nerve sheath tumors from other benign mesenchymal tumors such as fibrous histiocytomas, (b) cellular neurilemomas from malignant schwannomas, (c) malignant schwannomas from conventional fibrosarcoma (d) malignant melanomas from many carcinomas, and, possibly (e) juvenile xanthogranulomas from histiocytosis X.
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PMID:Value of S-100 protein in the diagnosis of soft tissue tumors with particular reference to benign and malignant Schwann cell tumors. 631 Feb 27


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