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)

Eighteen evaluable children with recurrent Langerhans' cell histiocytosis (LCH) which was resistant to standard therapy, were treated with etoposide (VP 16-213), 200 mg/m2/day for 3 days every 3 weeks, to study the efficacy and toxicity of this drug. Complete and partial responses were demonstrated in 15 patients (83.3%). Only one of the 12 children achieving a complete remission has relapsed. No dose-limiting major toxicities were registered. Although etoposide might be an effective treatment in recurrent LCH which needs a chemotherapeutic approach, it is emphasized that this drug must be used carefully.
Cancer 1988 Dec 15
PMID:Etoposide in recurrent childhood Langerhans' cell histiocytosis: an Italian cooperative study. 305 5

Congenital self-healing histiocytosis (CSHH) is a rare primary histiocytic skin disorder. Only a few cases have been studied by ultrastructure and immunohistochemistry. Here we report a new case that was investigated using an electron microscope and a panel of monoclonal (MCA) and polyclonal (PCA) antibodies. CSHH cells were found to bear the immunohistochemical phenotype of normal epidermal Langerhans cells (LC) and histiocytosis X (HX) cells (CD1a/c+, CD1b-, CD4+/-, human leukocyte antigen [HLA]-DR/DQ+, S-100+). However, an electron microscope showed a paucity of Birbeck granule (BG)-containing cells. This contrasted with their immunophenotype. This finding, along with other ultrastructural characteristics of CSHH cells, suggests that histologic differences exist between CSHH and HX. However, because no absolute histologic criterion is known that allows unequivocally the differential diagnosis between the two diseases, this distinction currently has to rely on clinical criteria, mainly the regressive course observed within a few months in CSHH. The precise nosologic position of CSHH among other histiocytic syndromes remains unsettled.
Cancer 1988 Feb 01
PMID:Congenital self-healing histiocytosis (Hashimoto-Pritzker). An ultrastructural and immunohistochemical study. 327 80

The effect of therapeutic ultraviolet light (UV) radiation on the skin lesions of histiocytosis X (HX) was studied in two old patients. Histiocytosis X cells in the biopsy specimens were strongly reactive with OKT-6, OK-Ia1, an anti-S-100 protein antibody, and were weakly stained with Leu-3a. Some HX cells outgrown from the explants bore immunoglobulin G receptors (Fc-IgG) and C3 receptors. In accordance with clinical improvement after repeated topical 8-methoxypsolaren (8-MOP) plus UV-A (PUVA) or UV-B radiation, the density of infiltrating HX cells gradually was decreased. The PUVA therapy seemed to be more effective than UV-B radiation in our treatment schedule. Even after repeated phototherapies, however, the reactivity of surface and cytoplasmic antigens related to OKT-6, OK-Ia1, and S-100 protein in the remaining HX cells were the same as in untreated HX cells. Although the exact mechanism remains obscure, satisfactory therapeutic results were obtained in response to the phototherapies. New skin lesions eventually recurred after cessation of the treatments, but such eruptions resolved when additional PUVA was resumed. These studies confirm that HX cells share a battery of cytologic characteristics with epidermal Langerhans' cells (LC) and that repeated phototherapies provide a beneficial effect for skin lesions of HX without adverse reactions.
Cancer 1986 May 15
PMID:The effect of phototherapies on cutaneous lesions of histiocytosis X in the elderly. 348 67

Primary bone tumors in early childhood are uncommon, and the incidence of malignancy in the 1st decade of life has not been evaluated adequately in the literature. For this reason, we conducted a retrospective review of 268 biopsies of the bone in 249 patients who were less than 10 years of age. Benign tumors were found much more frequently than malignant lesions. Osteochondroma and histiocytosis X were the most common lesions. The incidence of several benign lesions in our series was deceptively low, as many lesions showed typical radiographic findings, precluding the need for biopsy or excision. Of 41 primary malignant bone tumors, Ewing sarcoma was slightly more common than osteosarcoma. The rib was the most frequent site of the former; the distal femur, of the latter.
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PMID:Primary lesions of bone in the 1st decade of life: retrospective survey of biopsy results. 348 12

All cases diagnosed in Finland as non-Hodgkin's lymphoma (NHL), Hodgkin's disease or histiocytosis X in children younger than 15 years in 1953 to 1973, according to the Finnish Cancer Registry, were reexamined histologically. Only 55% of the cases originally diagnosed as NHL were regarded as such at reexamination. The others were mainly malignant nonlymphatic tumors such as neuroblastoma and different kinds of sarcomas. Seventy-two NHLs were diagnosed in 50 boys and 22 girls. The corrected age-specific incidence rate was 0.32/10(5). The most common histologic types were Burkitt's lymphoma (BL) (30 cases), lymphoblastic lymphoma (LBL) (26), large cell lymphomas (LCL) (six), and non-Burkitt's lymphoma (n-BL) (three). There were marked differences between BL and LBL in the course of the disease: BL was extranodal in 83%, LBL only in 4% (mediastinum was regarded as nodal); BL showed initial abdominal or pelvic involvement in 60% whereas LBL showed none; BL had initial mediastinal involvement in 7%, and LBL had it in 62%; all patients with LBL died whereas 23% of those with BL survived. Other types of NHL resembled BL in their course of disease. Patients with initial tonsillary involvement appeared to have the best prognosis and patients with mediastinal involvement the poorest. The importance of accurate histologic classification is emphasized. It appears to be most important to differentiate LBL from other types of NHL.
Cancer 1987 May 15
PMID:Non-Hodgkin's lymphomas in childhood. A clinicopathologic and epidemiologic study in Finland. 349 36

A 60-year-old man with a history of diabetes insipidus presented with a left groin mass, leg edema, and retroperitoneal adenopathy. Biopsy results of the involved lymph nodes were typical of eosinophilic granuloma. The patient was treated with etoposide and prednisone and had a complete regression of his lymphadenopathy and edema. This response suggests that etoposide may be a useful agent in the management of histiocytosis X.
Cancer 1986 Aug 15
PMID:Multifocal eosinophilic granuloma. Response of a patient to etoposide. 371 52

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.
Cancer 1985 May 15
PMID:A distinctive cutaneous malignant neoplasm expressing the Langerhans cell phenotype. Synchronous occurrence with B-chronic lymphocytic leukemia. 388 25

Solitary eosinophilic granuloma of the frontal lobe of the brain is a rare lesion. A 30-year-old Jordanian man with a history of recurrent bilateral chronic middle ear infections and tuberculosis presented with focal seizures. A solitary right frontotemporal mass was diagnosed by brain biopsy as an eosinophilic granuloma. Immunoperoxidase stains of virtually all histiocytes in the lesion were strongly positive for S-100. Four cases of eosinophilic granuloma and one of Letterer-Siwe disease were also examined, and virtually all histiocytes demonstrated strong S-100 positivity. In comparison, 24 reactive or infectious granulomas were examined for S-100 staining and most showed complete negativity of histiocytes in granulomas for S-100. This is the first report in the literature that has examined the results of S-100 staining in a large number of reactive or infectious granulomas and compared it with S-100 staining in eosinophilic granuloma. This report illustrates that S-100 can be useful in distinguishing eosinophilic granuloma, generally considered to be part of the larger group of histiocytic disorders known as histiocytosis X, from reactive or infectious granulomatous conditions of similar or confusing histology.
Cancer 1985 Jul 15
PMID:Primary eosinophilic granuloma of frontal lobe. Diagnostic use of S-100 protein. 389 Oct 65

Two infants presented with biopsy-proven histiocytosis X affecting multiple sites. Since neither showed evidence of organ failure or of constitutional upset, no specific therapy was given. In each case there was long-lasting spontaneous regression of disease. Analysis of blood mononuclear cells revealed a raised T4:T8 (helper:suppressor T lymphocyte) ratio at diagnosis but a normal ratio during remission. These observations support the argument that multi-system histiocytosis X, even in infants (Letterer-Siwe disease), is not a malignancy and that an "expectant" treatment policy may be indicated in selected patients.
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PMID:Spontaneous remission of multi-system histiocytosis X. 614 97


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