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)

Anti-Leu-3a, anti-Leu-3b, OKT4, and anti-T4 murine monoclonal antibodies react with a membrane component expressed by mature peripheral blood helper T cells and certain thymocyte subsets. Using a variety of immunologic staining techniques, we have demonstrated the reactivity of these antibodies with other cell types. Normal and neoplastic cells of monocyte/macrophage lineage bear the Ia+/Leu-6-/Leu-3+ phenotype, whereas histiocytosis X cells bear the Ia+/Leu-6+/Leu-3+ phenotype. The Ia+/Leu-6- cells of malignant histiocytosis and the Ia+/Leu-6+ epidermal Langerhans cells were variably Leu-3+. Normal monocyte/macrophage reactivity with anti-Leu-3/T4 appears to be primarily intracytoplasmic, whereas on U937 monocyte tumor cells, marked membrane reactivity is also observed. These results strongly suggest that certain cells other than helper T cells and thymocytes can express and, at least in some cases, synthesize a component previously regarded as T-lineage specific.
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PMID:Anti-Leu-3/T4 antibodies react with cells of monocyte/macrophage and Langerhans lineage. 640 71

A peculiar configuration of endoplasmic reticulum (ER) was observed by electron microscopy in some mononuclear cells from a malignant giant cell tumor of bone that had been removed from a cervical vertebral body in a 54-year-old female. The unusual ER consisted of looped, smooth tubules, which originated from poorly developed rough ER present in the tumor cells. The tubular structures were approximately 23 nm in diameter and contained paracrystalline material. The curvilinear tubular ER closely resembles the vermiform tubular structures that have been observed mainly in histiocytes from different clinical forms of histiocytosis X.
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PMID:An unusual form of endoplasmic reticulum in mononuclear cells of a giant cell tumor of bone. 653 68

A case of sinus histiocytosis with massive lymphadenopathy in a boy of 13 with multiple subcutaneous tumor-like formations 0.5 to 2.5 cm in diameter is described. The general condition of the patient was not changed despite the 7-month course of the disease. Mild anemia and increased ESR were observed. Microscopically the formations presented massive "dense" infiltrates of lymphoid, plasma cells, and histiocytes-macrophages with foci of fibrosis and xanthomatosis. Since the morphological picture of sinus histiocytosis with massive lymphadenopathy may to some extent imitate the substrate of some tumor diseases of the hemopoietic and lymphoid tissues (malignant histiocytosis, histiocytosis X, lymphogranulomatosis, lymphosarcoma), their differential diagnosis is presented.
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PMID:[Sinus histiocytosis with massive lymphadenopathy]. 660 46

Lymph node involvement by histiocytosis X is usually secondary to nearby bone or skin lesions. A patient with histiocytosis X confined to lymph nodes is rarely seen; however, we studied two such persons. In each, a lymph node biopsy was performed because the clinical aspects caused us to suspect neoplastic disease.
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PMID:Lymph node involvement by histiocytosis X. 697 50

A thyroid involvement is reported in a 10 year-old boy with histiocytosis X. It was revealed by goiter and hypothyroidism following diabetes insipidus and growth hormone deficiency. Hypothyroidism was exclusively due to the invasion of thyroid by the tumor, while the other endocrine changes were in favor of the histiocytic infiltration of hypothalamus. The exceptional feature of thyroid involvement in disseminated histiocytosis X is emphasized.
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PMID:[Thyroid involvement in histiocytosis X]. 698 32

For cancer therapy, especially radiation therapy, it is very important to determinate the invaded region. Since computed tomography (CT) is introduced into medical radiography, it has become well known that it has a highly diagnostic value not only for the intracranial lesions, but also for extracranial pathological processes. In this paper, 4 cases (pinealoma, rhabdomyosarcoma of the ear, nasopharynx tumor and Histiocytosis X of the orbit) are presented. In each case, pre-therapeutic and posttherapeutic CT images are shown. CT is also very useful during and after therapy in patients with malignancy of the head and neck.
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PMID:[Effectiveness of CT in the determination of the extent of infiltration of head and neck cancer]. 698 90

The computed tomographic (CT) findings of rhabdomyosarcomas involving the skull base in seven children are described. The tumor may arise in the nasopharynx, maxillary sinus, petrous bone, or orbit. Computed tomography clearly shows the full extent of bone destruction and replacement by soft tissue. The CT picture, however, is not specific and differential diagnosis with other aggressive lesions involving the base of the skull such as histiocytosis X, neuroblastoma, and lymphoma can be made only with a combined clinical and radiological approach.
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PMID:Computed tomography of rhabdomyosarcomas of the skull base in children. 706 11

9 cases of malignant histiocytosis (3 males, 6 females, age 30 to 83 years) have been observed in the biopsy and autopsy material of the Institute of General Pathology and Pathological Anatomy of the Medical Academy of Erfurt, GDR. Malignant histiocytosis which derives from tissue macrophages/histiocytes is characterized by a proliferation of non-cohesive atypical histiocytes mainly in lymph nodes, spleen, liver, and bone marrow. The tumor cells are large, pleomorphic and show an irregularly-shaped, cleaved and often kidney-like nucleus with 1-3 medium-sized nucleoli. The cytoplasm is moderately basophilic and grey-blue when stained with azure B eosin or according to Pappenheim. Diffuse cytoplasmic activities of nonspecific esterase and acid phosphatase are further properties. Another sign of malignant histiocytic cells should be stressed, i.e., the diffuse intracytoplasmic perinuclear PAS-positive spot which hitherto has not been mentioned in the literature. Morphological variants of malignant histiocytosis are fibrosing (2 cases) and erythrophagocytosing types (2 cases). Reactive histiocytosis, histiocytosis X and malignant non Hodgkin-lymphomas, esp. immunoblastic lymphomas, are to be taken into account in differential diagnosis.
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PMID:[Contribution to morphology of malignant histiocytosis (author's transl)]. 709 May 92

Ninety three cases of diabetes insipidus are reviewed after 1 to 20 years follow-up. A codified water deprivation test realized in 50 cases revealed 31 severe cases and 19 partial forms. Apart from surgical causes of diabetes insipidus (16%), tumors represent the most frequent etiology (33.5%), essentially craniopharyngiomas and pinealomas. Histiocytosis X (16%) is also an important cause. In one third no cause could be found. Decreased secretion of anterior pituitary hormones is very common in association with surgical and tumoral causes and can also be found in idiopathic forms. Thus, in our experience, detection of a pituitary deficit is not necessarily associated with a tumor. It can be pointed out that when an etiology is found it is always discovered during the four years following the onset of diabetes insipidus. This implies that very careful neurological and neuroradiological follow-up is necessary during this period.
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PMID:[ADH-deficient diabetes insipidus in children. A study of 93 cases (author's transl)]. 746 83

Although the first clinical description of Langerhans cell histiocytosis (LCH) was published over a century ago, the aetiology and pathogenesis of this enigmatic disorder are still remained unknown. Viral, immunological, neoplastic and other pathogenetic mechanisms have been considered, but none has been proven. The prevailing opinion is that LCH is a reactive disorder rather than a neoplastic process, but this presumption has never been definitively tested. A key feature of a neoplasm is its clonal derivation from a single cell. To determine if LCH is a polyclonal reactive or a clonal disorder, we and others have recently used molecular biological techniques to assess clonality in LCH. Using X chromosome-linked DNA probes that can detect clonal or polyclonal X chromosome inactivation patterns in female tissues, clonal CD1a+ histiocytes have now been detected in the lesional tissues in each of 16 females affected with LCH. Most of these patients were studied prior to the initiation of therapy. Lymphoid clonality was excluded in all cases in which it was studied, confirming that the clonal cells in LCH are the CD1a+ dendritic cells presumed to be pivotal in this disorder. Two distinct lesions (a pre-treatment bone biopsy and a lymph node biopsied 3 years later) have been studied in only one case to date; the same clonal pattern of X chromosome inactivation was observed, consistent with persistence of the same clone during this patient's disease course.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Detection of clonal histiocytes in Langerhans cell histiocytosis: biology and clinical significance. 752 Dec 1


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