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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Langerhans cell histiocytosis is currently regarded as a reactive proliferative process of Langerhans cells rather than a malignancy. The disease is characterized by Langerhans cell infiltration of skin, lung, bone and other organs. We report a 74-year-old man with Langerhans cell histiocytosis who had generalized hemorrhagic and crusted papules. He also had diabetes insipidus. Because he did not have any severe constitutional symptoms or failure of vital organs, we applied topical PUVA treatment to his skin lesions, which responded well to the therapy. Diabetes insipidus, however, remained, in spite of X ray radiotherapy for the pituitary lesion.
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PMID:Satisfactory remission achieved by PUVA therapy in Langerhans cell hisiocytosis in an elderly patient. 872 Feb 57

Langerhans cell histiocytosis is an infrequent and enigmatic proliferative disorder that commonly presents in the head and neck region. This is an analysis of 77 patients with Langerhans cell histiocytosis treated at Children's Hospital and Dana Farber Cancer Institute from 1974 through 1993. The study focused on clinical findings, anatomic location and extent of disease, therapy, and outcome. The patients were, on average, under 5 years of age at initial presentation. Over 62 percent of the patients had signs and symptoms referred to the craniofacial skeleton. Osteolytic lesions of the cranium were the most common, followed, in frequency, by scalp rash, osteolytic mandibular tumor(s), enlarged nodes, and gingival swelling or ulceration. Single bony lesions usually were treated with curettage or radiotherapy. Chemotherapy was used commonly for advanced disease with multifocal or disseminated presentation. Initial therapy included moderate doses of single agents; other agents were added if no response was achieved. The natural history of Langerhans cell histiocytosis varied from an acute fulminant course, a waxing and waning chronic disease, to spontaneous regression. Young age at presentation and organ dysfunction predicted a poor prognosis. Statistical analysis showed that there was no significant relationship between outcome and extent of skeletal involvement when controlling for age or organ dysfunction.
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PMID:Langerhans cell histiocytosis: an uncommon disease commonly manifesting in the craniofacial skeleton. 876 8

Two cases of Langerhans cell histiocytosis (LCH) expressing as Hand-Schuller-Christian syndrome with diabetes insipidus, hyperprolactinemia and empty sella are here reported. Up-to-date this four-fold association is lacking in world literature and it is here discussed in the light if LCH is a cancer or the clinical expression of an immunologic disorder.
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PMID:Langerhans cell histiocytosis, diabetes insipidus, hyperprolactinemia and empty sella: a four-fold association. Report of two cases. 885 91

We have studied the reactivity patterns of a previously described pan-macrophage monoclonal antibody (MAb) D11 in 324 cases of acute leukemia and malignant lymphoma (ML). Reaction of D11 in tissue sections was restricted to histiocytes and macrophages. In non-Hodgkin's ML, D11 helped to confirm or to establish the histiocytic nature in 8 of 96 cases, i.e., in 4 of 6 histiocytic MLs; 2 of 13 anaplastic large-cell lymphomas; 1 of 4 large-cell immunoblastic clear-cell MLs; and 1 of 2 histiocytosis X cases. Positive reaction of D11 in acute lymphoblastic leukaemia (ALL) was found in 9 of 86 cases (all belonging to early B-lineage leukemia), of which 4 were CD34-positive and 5 co-expressed 1 or more myeloid/monocytic antigens. MAb D11 did not react in 42 cases of acute-myeloblastic-leukemia (AML) FAB variants M0-M5, except 1 acute mixed-lineage leukemia M1/pre-pre-B. Comparative study of the MAb D11 and a standard CD68 MAb KP- 1 showed that the antigens belong to different epitopes of different molecules.
Int J Cancer 1996 Oct 09
PMID:Reactivity of anti-macrophage monoclonal antibody D11 in human leukemia and malignant lymphoma. 890 Apr 21

Cytological, immunohistological and electron microscopical observation of 21 percutaneous fine needle punctures of retroperitoneal, pelvic and abdominal lymph nodes after borderline lymphography and computer tomography and 6 punctures of tumours after tomography allowed classification of primary metastases from the small pelvis in 14 patients and characterized tumours in 4 patients, which could not be demarcated by sonography. We distinguished yolk sarcoma metastasis, prostate gland cancer metastasis, three cases of nodular metastases of seminoma cells, and two metastases of melanoma. Malignant cells of Hodgkin's lymphogranuloma and non-Hodgkin's lymphoma were distinguished in seven samples of fine needle puncture. We found malignant cells of adenocarcinoma, T-immunoblastoma, pancreas carcinoma and histiocytosis X in four punctures of tumours. Fine needle puncture processed for electron microscopy with buffered fixation and harvested into Lowicryl K4M resin through centrifugation makes it possible to detect even the minimum of cells present, preserves the structure of cells and enables to correlate cytological findings in semithick sections with correspond ultrastructure in followed series of semithin sections.
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PMID:Malignant cells revealed in fine needle punctures of lymph nodes and tumours by electronmicroscopical methods. 890 20

The International Classification of Childhood Cancer (ICCC) updates the widely used Birch and Marsden classification scheme. ICCC is based on the second edition of the International Classification of Diseases for Oncology (ICD-O-2). The purpose of the new classification is to accommodate important changes in recognition of different types of neoplasms, while preserving continuity with the original classification. The grouping of neoplasms into 12 main diagnostic groups is maintained. The major changes are: (1) intracranial and intraspinal germ-cell tumours now constitute a separate subgroup within germ-cell tumours; (2) histiocytosis X (Langerhans-cell histiocytosis) is excluded from ICCC; (3) Kaposi's sarcoma is a separate subgroup within soft-tissue sarcomas; (4) skin carcinoma is a separate subgroup within epithelial neoplasms; (5) "other specified" and "unspecified" neoplasms are now usually separate sub-categories within the main diagnostic groups. Draft copies of the ICCC were distributed to some 200 professionals with interest and expertise in the field and their comments are considered in this final version. This classification will be used for presentation of data in the second volume of the IARC Scientific Publication "International Incidence of Childhood Cancer." A computer programme for automated classification of childhood tumours coded according to ICD-O-1 or ICD-O-2 is now available from IARC.
Int J Cancer 1996 Dec 11
PMID:The international classification of childhood cancer. 898 Jan 80

There is little information available regarding epidemiologic risk factors for Langerhans cell histiocytosis (LCH). An exploratory investigation was conducted using information obtained from parents of 177 cases of LCH diagnosed before 21 years of age (median 2 years). Utilizing data available from the Children's Cancer Group, LCH cases were compared to two matched control groups including 614 patients diagnosed with a variety of childhood cancers and 318 community controls. Questionnaire data included information on demographics, prenatal and perinatal factors, complications in the neonatal period, environmental exposures, family medical history, and childhood exposures. Factors found to be statistically significantly associated with an increased risk of LCH included: maternal urinary tract infection during the index pregnancy, feeding problems during infancy, and blood transfusions during infancy. Use of supplemental vitamins was associated with a significantly decreased risk of LCH. Results from this exploratory study provide a basis for speculation on potential etiologic risk factors for LCH. Future epidemiologic investigations of LCH need to consider the presenting disease characteristics in assessing possible etiologic factors.
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PMID:Langerhans cell histiocytosis: an exploratory epidemiologic study of 177 cases. 898 44

Histiocyte disorders are characterised by tissue infiltration with cells of monocyte/macrophage lineage, with two disorders, Langerhans' cell histiocytosis (LCH) and haemophagocytic lymphohistiocytosis (HLH) accounting for the overwhelming majority of cases in childhood and, apart from monocyte variants of acute myeloid leukaemia, histiocytic malignancy is very rare. Although both LCH and HLH are considered reactive disorders, the prognosis of these conditions differs greatly, LCH is usually self limiting, with a mortality of 10%, but HLH is usually fatal, with a mortality of over 80% in the absence of bone marrow transplantation. Increased levels of cytokines have been demonstrated in these disorders, and may be responsible for many of the clinical features: it is unclear whether histiocytes themselves, or other immune cells, particularly T lymphocytes, are the abnormal cell population. Due to the rarity of histiocyte disorders, collaborative studies are essential to improve understanding and advance treatment.
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PMID:Histiocyte disorders. 903 34

To estimate the risk of secondary leukemias after treatment with etoposide (VP-16), we evaluated subjects treated for Langerhans' cell histiocytosis (LCH) according to cooperative protocols in Italy or in Austria, Germany, Holland and Switzerland (AGDS). For each subject, information was collected on the cumulative dosages of chemotherapy and radiotherapy received, vital status and occurrence of secondary leukemia. The expected number of leukemias was estimated using age-specific incidence rates from the cancer registries in Italy and Germany. Standardized incidence ratios (SIR) were used to measure the risk of secondary leukemia among LCH patients. Five leukemias occurred among the 241 Italian study patients (SIR 520), whereas no cases were reported among the 363 AGDS patients. Interestingly, and in contrast to previous descriptions of epipodophyllotoxin-related leukemias which are mostly FAB M4 or M5, these leukemias showed typical FAB M3 features, and received a dose of VP-16 > 4,000 mg/m2. Among the AGDS cohort, very few subjects were exposed to high doses of VP-16. The risk of secondary acute non-lymphoblastic leukemia (s-ANLL) among the Italian subjects exposed to VP-16 was more than 1,000 times greater than expected. The study suggests that high doses of VP-16 appear to increase the risk of s-ANLL in LCH patients. The fact that all the leukemias described in the Italian LCH cohort were promyelocytic, and evidence of a higher incidence of promyelocytic leukemias among Italians and Latinos, suggest that high doses of etoposide in subjects of Latino origin may lead to aberrations on chromosomes 15 and 17.
Int J Cancer 1997 Mar 28
PMID:Risk of secondary leukemia after treatment with etoposide (VP-16) for Langerhans' cell histiocytosis in Italian and Austrian-German populations. 909 58

Seven cases of breast involvement by extranodal Rosai-Dorfman disease are presented. The patients were women and their ages ranged from 15 to 84 years. Three patients had disease confined to the breast; one had involvement of the breast and ipsilateral axillary lymph nodes, and two had bilateral breast involvement as well as disseminated systemic disease. In all cases the clinical and radiographic presentation of the breast lesion raised the possibility of a malignant tumor. All but one of the lesions were treated by excisional biopsy. Microscopically, the lesions were relatively circumscribed, often multinodular masses, located in the breast stroma, with or without associated involvement of the subcutaneous tissue and dermis. They were composed of sheets of S-100 protein-positive large histiocytes displaying lymphocytophagocytosis, scattered in a polymorphous background of mature lymphocytes and plasma cells. The microscopic differential diagnosis includes idiopathic granulomatous mastitis, infective granulomas, Langerhans' cell histiocytosis, Erdheim-Chester disease, fibrous histiocytoma, and malignant melanoma.
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PMID:Breast involvement by extranodal Rosai-Dorfman disease: report of seven cases. 1007 33


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