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 light-microscopic and ultrastructural findings in a case of so-called giant cell tumor of soft parts, localized at the dorsal side of the left foot of a 23-years-old male are described. An amputation of the lower extremity was performed and subsequently chemotherapy with adriamycin was given for 3 months. Despite the histology and cytologic malignant appearance and the evident vascular invasion, already present at the time of the first excision, the last known status 2 years later seems favorable. There are no pathologic lymph nodes in the groins and no signs of metastases on chest X-rays. From the electron-microscope study no definite conclusion can be drawn as regards the histogenesis of this tumor. we feel, as do others, that many of the principal mononuclear tumor cells are poorly differentiated mesenchymal cells. Some of the neoplastic cells, however, show ultrastructural features suggestive of chondro- or osteoblasts (a well-developed r.e.r. containing electron-dense material; multiple Golgi complexes; masses of glycogen; interdigitating cell membrane villi; cytoplasmic filaments; an extracellular amorphous matrix). Some of the larger tumor cells have the submicroscopic aspects of histiocytes as described in osseous, cutaneous, or pulmonary lesions of the histiocytosis X group. As yet undetermined cytoplasmic inclusion bodies constitute another rare observation in our material.
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PMID:Giant cell tumor of soft parts. An ultrastructural study. 18 82

After reviewing the syndrome of histiocytosis X, the special problems of an attack on the eye are discussed with regard to clinical, histological and radiological findings. By means of two cases, all aspects important for the diagnosis and treatment of histiocytosis X are discussed with reference to the literature. A histologically verified histiocytosis X in the area of the orbita is described in an almost 2 year old girl and another girl aged 7 years and 3 months. In both cases, the disease began with an edema of the upper eyelid and swelling of the lateral orbital margin. The second case showed in addition the clinical symptoms of a dacryoadenitis. The X-ray pictures of the skull revealed a large triangular osseous lesion in the area of the orbital margins. Blood chemistry, hematological tests and the bonemarrow puncture did not yield any pathological findings. The diagnosis of histiozytosis X could be made only by a biopsy from the tumor area. The swelling in the area of the eyelids and the lateral orbital margin disappeared under cytostatic therapy with Vincristin and Prednison.
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PMID:[Histiocytosis-X of the orbit (author's transl)]. 108 43

The monoclonal antibody Ki-M1P recognizes a formalin/paraffin-resistant differentiation epitope of monocytes and their macrophage derivatives [Radzun et al., Lab Invest 65:306, 1991]. To evaluate its usefulness for neuropathology, we examined a variety of routinely processed tissues using immunohistochemistry. In normal brains, positivity was restricted to ramified microglial cells. Intense labeling of macrophages, ramified and ameboid microglial cells, and rod cells was seen in brains with various degenerative and inflammatory disorders. Astrocytes were negative as determined by double-immunofluorescence labeling using Ki-M1P and anti-glial fibrillary acidic protein (GFAP). Histiocytic lesions (histiocytosis X, xanthogranulomas, granulomatous inflammation) were immunopositive. Among 107 tumors, reactivity of Ki-M1P was observed with some schwannoma and meningioma tumor cells. In addition to macrophages, most gliomas contained small, elongated Ki-M1P-positive cells, which were negative for GFAP. Positivity was also found in two glioblastoma cell lines. Immunoblotting performed on spleen, meningioma and glioblastoma specimens revealed one to three bands in the range of 110 to 130 kDa. We conclude that Ki-M1P can serve as a reliable marker for brain macrophages and microglial cells in routinely processed normal and non-neoplastic tissues, whereas due to the unexpected immunoreactivities results obtained with neoplastic tissues should be carefully interpreted.
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PMID:Ki-M1P as a marker for microglia and brain macrophages in routinely processed human tissues. 146 66

Four cases of benign Langerhans cell histiocytosis limited to the skin were studied. In all three self-healing cases (cases 2, 3, and 4) many dense bodies, myelin bodies, and worm bodies were found. In one chronic case (case 1) none of these was identified. In all four cases, in addition to CD1, HLA-DR, and S-100 stains, interferon-gamma and S-100 beta-subunit were positive in the dermal tumor cells. Both interferon-gamma and S-100 beta-subunits were negative in the normal epidermal Langerhans cells. A comprehensive literature review yielded 87 cases of skin-limited Langerhans cell histiocytosis. These cases could be subgrouped into three categories: (1) those that resolved spontaneously, (2) those that responded to therapy and had no recurrence, and (3) those with persistent or recurrent lesions, not responding to therapy but still limited to the skin.
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PMID:Immunohistochemistry and electron microscopy in Langerhans cell histiocytosis confined to the skin. 168 9

Between 1987 and 1991, 248 long-term survivors of childhood cancer have been studied at the Oncology Unit of the Children's Hospital of Buenos Aires. The tumors were diagnosed between 1965 and 1986 as, retinoblastoma: 65, lymphoma: 57, nephroblastoma: 36, neuroblastoma: 25, germ-cell tumors: 18, sarcoma: 19, bone tumors: 7, lymphoepithelioma of cavum: 4, histiocytosis X: 9, others: 8. The treatment consisted of, surgery: 25, surgery+chemotherapy: 40, surgery+radiotherapy: 8, chemotherapy: 23, chemotherapy+radiotherapy: 42, and surgery+chemotherapy+radiotherapy: 110. There are alive without evidence of cancer disease 234 survivors between 5 and 25 years after diagnosis. Severe organic disabilities were observed in 181 survivors and moderate in 142. Thirteen patients died because of second malignant neoplasia and 1 patient with lung metastasis 9 years after diagnosis of nephroblastoma. In 180 survivors the data of instruction was available. Seventy participate in sports and 13 in artistic activities. Eight survivors are university graduates and 24 are employees. Five young women were mothers and one young man was a father. The meaning of concept of "cure" is discussed from the point of view of the physicians and the survivors. In order to detect deleterious late effects of cancer and their treatment the follow-up must be continuous.
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PMID:[The concept of cure in children with cancer]. 182 19

Sixty-two patients with histiocytosis X were followed for an average of 5 years. The patients were classified into three groups: general visceral types (14 cases), multiple eosinophilic granulomas (nine cases), and solitary eosinophilic granulomas (39 cases). One hundred bony lesions were noted in 60 of the 62 patients. The bone lesions showed progressive improvement in single and multiple eosinophilic granulomas independent of treatment type. After biopsy, patients received no treatment unless there was a dangerous extension into the soft tissues because of its site, i.e., in the skull. In the general visceral types, chemotherapy was effective in visceral sites and in extensions of the tumor outside the bone but did not alter the natural history of the bony lesion.
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PMID:Bone lesions in histiocytosis X. 186 Sep 45

Eosinophilic granuloma is a solitary form of histiocytosis X with a usually good prognosis. Sometimes spontaneous healing can even occur. The authors report a case of a 30-month-old boy who presented an eosinophilic granuloma of the lateral orbital wall. The correct diagnosis was obtained after computed tomographic scan followed by fine needle aspiration biopsy with histopathologic examination. The tumor increased rapidly, including a medial displacement of the globe and limitation of the eye movement. So chemotherapy (Vinblastine) with corticotherapy was performed. The clinical course was favorable, with no evidence of recurrence or systemic involvement. The originality of this case was the young age of the patient, the course and the efficacy of treatment. The authors emphasize the management of the disease.
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PMID:[Solitary eosinophilic granuloma of the external wall of the orbit]. 191 25

The interstitial lung diseases are comprised of a group of pulmonary disorders characterized clinically by diffuse infiltrates on the chest radiograph and histologically by distortion of the gas exchanging portion of the lung. The physiologic correlates are restriction of lung volumes and impaired oxygenation. The term "interstitial" when applied to these diseases is actually a misnomer because it implies that the inflammatory process is limited specifically to the area between the alveolar epithelial and capillary endothelial basement membranes. The diseases currently grouped as "interstitial" also frequently involve the alveolar epithelium, alveolar space, pulmonary microvasculature, and less commonly, the respiratory bronchioles, larger airways, and even the pleura. The enormous differential diagnosis of interstitial lung disease can be made manageable by understanding that pneumoconiosis, drug-induced disease, and hypersensitivity pneumonitis account for over 80% of the responsible entities and can usually be identified from the patient's history. The nine remaining diseases/disease categories include: sarcoidosis, idiopathic pulmonary fibrosis, bronchiolitis obliterans-organizing pneumonia, histiocytosis X, chronic eosinophilic pneumonia, collagen vascular disease-associated interstitial lung disease, granulomatous vasculitis (Wegener's granulomatosis, Churg-Strauss syndrome, lymphomatoid granulomatosis), Goodpasture's syndrome, and pulmonary alveolar proteinosis. The diagnosis of a specific interstitial lung disease can be made via various means including the patient's history, specific serologies, bronchoalveolar lavage, transbronchial biopsy, and biopsy of extrathoracic tissues or open lung biopsy. A directed diagnostic approach can be formulated based on an understanding of these techniques and a thorough knowledge of the clinical presentations and specific diagnostic criteria for each of the major diseases. This monograph will serve as a guide for the clinician to use in evaluating and treating patients with interstitial lung disease. We begin by reviewing the clinical presentation, diagnostic criteria, and management of specific interstitial lung diseases excluding pulmonary infection, neoplasm, and sarcoidosis. Pneumoconiosis and drug-induced syndromes are not discussed in detail, but the agents responsible and pertinent exposures are presented in tabular form in the discussion of the general diagnostic approach.
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PMID:Interstitial lung disease. 199 45

Langerhans cell histiocytosis (LCH) of the female genital tract is rare. Four new cases are reported, and there is a review of the 38 cases in the literature. This disease may involve the vulva, vagina, cervix, endometrium, and ovary. Four distinct patient groups, segregated on the basis of initial presentation and subsequent anatomic extent of disease, were categorized as follows: (1) "pure" genital LCH, (2) genital LCH with subsequent multi-organ involvement, (3) oral or cutaneous LCH with subsequent genital and multi-organ involvement, and (4) diabetes insipidus with subsequent genital and multi-organ disease. Although involvement of the genital tract can occur at any age, it is most common in young adulthood. Clinically, LCH may mimic either primary neoplasia or various inflammatory lesions; the major pathologic differential diagnosis is venereal and other inflammatory diseases. The pure genital form may have a distinct nosologic position in the spectrum of LCH similar to the "pure," self-limited cutaneous histiocytosis seen in infants. There is no correlation between histologic findings and the outcome of the genital lesions. There is also no correlation between clinical presentation and/or the extent of involvement and outcome of genital lesions; complete regression, partial improvement, persistent lesions, and recurrences were seen in all four groups of patients. The treatment of genital LCH is not well defined and is highly individualized. Therapy has included surgery, radiation, topical corticosteroids, topical nitrogen mustard, systemic chemotherapy, and combination therapy; mixed results were obtained with all treatment modalities. Although no modality has been shown to yield a superior outcome, complete surgical excision is advocated as initial therapy.
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PMID:Langerhans cell histiocytosis of the female genital tract. 200 54

The endoscopic examination of the tracheobronchial tree is most helpful in the diagnosis and staging of bronchial carcinoma. Tumors that are endoscopically visible may be confirmed in more than 95% of the cases. In localized peripheral tumors, the diagnostic yield of bronchoscopy is significantly lower; for peripheral metastases, only about 10%. In diffuse interstitial pulmonary diseases other than malignancies, some infections, and histiocytosis X, bronchoscopy including transbronchial biopsy is less successful.
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PMID:The diagnostic yield of bronchoscopy. 204 23


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