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

VP-16-213, a semisynthetic podophyliotoxin, was tested for antitumor and clinical toxicity in 126 children. The drug was administered iv daily x 5 days every 2 weeks at a starting dose of 75 mg/m2/day. The dose was increased by 25 mg/m2/day/course until clinical response or significant toxicity occurred. The only major toxicity was hematologic, with neutropenia as the most predominant feature. There was one local allergic reaction at the site of injection. No systemic allergic responses were reported. The drug demonstrated significant activity in acute myelomonocytic leukemia with four responses among 19 patients, less activity in acute myelocytic leukemia with two responses among 44 patients, and little activity in acute lymphocytic leukemia with only one partial response among 12 patients. Objective partial responses occurred in ten of 48 patients with solid tumors: two each with Wilms' tumor, lymphoma, and histiocytosis X, and one each with rhabdomyosarcoma, neuroblastoma, Ewing's sarcoma, and undifferentiated carcinoma. The inclusion of VP-16-213 in combination chemotherapy for childhood acute myelomonocytic leukemia and acute myelocytic leukemia appears indicated in patients relapsing after initial therapy. For solid tumors this is an interim report, with further patient accrual required before specific comments can be made.
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PMID:Phase II study of VP-16-213 in childhood malignant disease: a Children's Cancer Study Group Report. 29 6

Five cases of bronchogenic carcinoma were observed among 93 patients with pulmonary histiocytosis X (Hx). Mean age at the time of diagnosis of Hx was 42 years; on the average, cancer occurred 10.5 years later. All patients were smokers and continued to smoke heavily at the time of diagnosis of cancer. Comparison of the five cases associating Hx and lung carcinoma with a group of 88 control patients suffering from Hx alone suggested that smoking played the predominant role in the pathogenesis of cancer. In fact, among the four patients with Hx and carcinoma older than 45 years, tobacco consumption was significantly greater (64.7 +/- 37 pack-year, mean +/- SD) than that of the 15 control patients of the same age with only Hx (40.8 +/- 11.6, p less than 0.01). In light of this good correlation, the diagnosis of Hx strongly advocates stopping tobacco smoking and long-term medical follow-up.
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PMID:Bronchogenic carcinoma in patients with pulmonary histiocytosis X. 160 Jul 81

Between 1985 and 1989, 395 patients with primary lung cancer were treated at the Percy military teaching hospital, Paris. Among them were 16 patients (4%) aged between 29 and 40 years (mean: 34 years) and smokers (mean cumulative tobacco consumption 24 P.A.). Six of these (37%) had lung parenchyma dystrophy with bilateral apical bullae of emphysema, 1 had a clean cavity left by a previous lung abscess and 1 had microcytic fibrosis resulting from histiocytosis X of the lungs and bones. In all cases cancer had developed in contact with bullous lesions or sequelae. Cancer was discovered during radiological mass screening in 6 cases (37.5%), on the finding of systemic or thoracic symptoms in 5 cases and because of a distant metastasis in 5 other cases. Pathological examination revealed an adenocarcinoma in 8 out of 16 patients and only one small-cell carcinoma. Nearly two-thirds of the patients had reached an advanced stage: 3 were in stage III A, 1 in stage III B and 6 in stage IV. Eight patients underwent curative surgery (7 lobectomies, 1 pneumonectomy). Survival was known with precision in 14 patients: 8 died after a mean follow-up of 15 months (range: 3 and 31 months); 3 were alive with an active cancer and 4 are still alive in complete remission after curative surgery. Six published studies totalling 387 cases are concordant in demonstrating that primary lung cancer is severe in adults below 40 years of age and that surgery is useful in such cases, even with N2 lymph node involvement.
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PMID:[Primary bronchial cancer in subjects aged 40 or younger]. 166 14

The cytologic and histologic findings are reported in a case of papillary carcinoma arising within a thyroid gland pathologically enlarged by histiocytosis X. Fine needle aspiration (FNA) biopsies of a thyroid nodule in a patient with longstanding histiocytosis X produced a scanty amount of colloid, a moderately dense mixed inflammatory infiltrate and numerous small papillary fragments lined by cuboidal-to-columnar cells. Both the inflammatory cells and the epithelial cells showed nuclear grooves; the two populations of neoplastic cells were distinguished, and the correct diagnosis of the nodule was made, by recognizing the greater amount of granular cytoplasm of the mononucleated Langerhans' cells. The rare involve-of the thyroid by histiocytosis X is reviewed; this case, which appears to be the first reported instance of the co-occurrence of histiocytosis X and papillary carcinoma of the thyroid, indicates that patients with histiocytosis X should be observed for the development of thyroid carcinoma and that FNA biopsy can make the distinction between the two conditions.
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PMID:Thyromegaly secondary to simultaneous papillary carcinoma and histiocytosis X. Report of a case and review of the literature. 192 75

The case of a 28-year-old woman with histiocytosis X confined to the thyroid is described. A partial thyroidectomy specimen from this patient was at first interpreted as showing poorly differentiated thyroid carcinoma. Subsequent investigations, including electron microscopy, revealed the true nature of the lesion. Even on retrospective examination, the two conditions can simulate each other very closely and may be easily confused.
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PMID:Histiocytosis X of the thyroid masquerading as thyroid carcinoma. 325 46

Twenty malignant T-cell lymphomas are described in which the initial histological diagnosis was equivocal. Among lymphoma entities initially falsely interpreted on pure morphological grounds were Hodgkin's disease, lymphoepithelioid cellular lymphoma, immunoblastic lymphoma with epithelioid cells, malignant histiocytosis, histiocytosis X, centroblastic lymphoma, undifferentiated carcinoma, and undifferentiated tumour. Variations in the expected mean survival of initially diagnosed tumours and T-cell lymphomas confirm the need for immunohistological cell typing in lymphoma classification.
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PMID:Classification and differential diagnosis of 20 problem cases of occidental malignant T-cell lymphomas. 348 36

Langerhans cells represent a minor epidermal cell population in mammals. They are also observed in squamous epithelia of the oesophagus, vagina and cervix, as well as in oral epithelia. They occur in higher density in the non keratinized epithelium. These cells are characterized by a dendritic pattern, a clear cytoplasm and ultrastructurally by the presence of Birbeck granules. They are usually located in a suprabasal position. Their bone marrow origin is now well established. Surface markers and functional properties identify them as belonging to the macrophage/monocyte lineage. Langerhans cells can be identified in tissue sections by immunofluorescence or immunoperoxidase techniques using monoclonal antibodies directed against surface antigens such as class II histocompatibility antigens, T6 marker, or possibly T4 marker. There is also a cytoplasmic marker, the S-100 protein. A renewed interest in Langerhans cells comes from evidence of their role in the cutaneous immune response. At present these cells are considered as dendritic cells expressing a high density of class II histocompatibility antigens and behave as very potent antigen presenting cells that activate mainly helper T lymphocytes. However, experimental data on antigen processing and interleukin 1 secretion are still lacking. This review also examines the oral pathology literature with respect to modifications in the number or localization of Langerhans cells and their proximity to T lymphocytes, for example in lichen planus, Behcet's syndrome, erythema multiforme, gingivitis and oral carcinoma. Histiocytosis X represents a particular case in which the Langerhans cell itself is affected.
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PMID:[Physiology of Langerhans cells and their potential role in oral pathology]. 351 86

Malignant lymphomas of the skin, excluding mycosis fungoides, are pathologically, immunologically, and clinically heterogeneous. Varying patterns and degrees of cutaneous infiltration are encountered in all histologic subtypes of non-Hodgkin's lymphomas. Immunologic studies have shown relatively equal numbers of cases with B and T cell phenotypes, but true histiocytic lymphomas of the skin also occur. Patients may be of any age, and they may have lymphoma in any clinical stage. A low clinical stage and a low-grade histologic subtype are significant factors for long survival. The differential diagnosis includes a variety of lymphoid infiltrates that are referred to as a group as cutaneous lymphoid hyperplasia; it also includes lymphomatoid papulosis as well as nonlymphoid cutaneous infiltrates such as myeloid leukemias, histiocytosis X, malignant histiocytosis, regressing atypical histiocytosis, and neuroendocrine (Merkel) cell carcinoma. Distinction of cutaneous lymphomas from these entities requires comprehension of multiple variables, including immunologic as well as morphologic and clinical factors.
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PMID:Malignant lymphomas of the skin: their differentiation from lymphoid and nonlymphoid cutaneous infiltrates that simulate lymphoma. 391 53

Fine needle aspiration biopsy was carried out in 35 patients with vertebral lesions in an attempt to differentiate between histiocytosis X and spondylitis. In 7 patients (6 with histiocytosis X and 1 with carcinoma) aspiration biopsy was of decisive diagnostic value. In 26 cases aspiration biopsy supported the diagnosis of spondylitis and in 2 patients no diagnostic information was obtained by aspiration biopsy. In selected cases of bone lesions in the spine, fine needle aspiration biopsy is recommended as an easy and important diagnostic procedure.
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PMID:Histiocytosis X. IX. Fine needle aspiration biopsy for differentiation between histiocytosis X and spondylitis. 632 May 92

Advances in pathology allow for more specific diagnoses of orbital disease. The authors discuss the value of awareness of advances in cytology, histochemistry, immunohistochemistry and electron microscopy as applied to orbital disease. Modern cytologic technique can aid in clearer visualization of cellular detail with improved diagnosis of thin needle aspiration biopsies. Histochemistry offers an increasing range of methods for identification of cellular and extracellular substances such as amyloid, fibrin, neuroglia, and collagen. Immunohistochemistry allows for identification of an ever-increasing number of component antigens including immunoglobulins, myoglobins, keratin, glial fibre protein, etc. Electronmicroscopic technique including plastic embedding allow for specific identification of lesions based on subcellular components and characteristic nuclear, cytoplasmic, membrane, basement membrane and stromal components. The value of these methods has been demonstrated with case presentations of "small round cell tumors" of the adult and child. In addition, the pathologic diagnosis of several rare lesions of the orbit including neuroendocrine carcinoma, histiocytosis X, simultaneously occurring poorly differentiated mucoepidermoid carcinoma of lacrimal gland and adenocarcinoma of the prostate are demonstrated to underline the advances in technology. Emphasis is placed on the management of biopsy material to maximize diagnostic potential.
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PMID:Recent advances in pathology as applied to orbital biopsy. Practical considerations. 637 43


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