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)

Using a monoclonal antibody specific to the Lewis X antigen (anti-Lex), the authors studied 103 cases of Hodgkin's disease (HD) in comparison with 57 cases of non-Hodgkin's lymphoma (NHL); three cases of granulocytic sarcoma (GS); two cases of malignant histiocytosis (MH); one case of monoblastic leukemia (ML); one case of interdigitating reticulum cell sarcoma (IRCS); six cases of histiocytosis X (HX); one case of reticulohistiocytoma (RH); 44 various reactive conditions of the lymph node (LN). Reed-Sternberg and related (R-S) cells stained selectively in 80 of 92 cases of HD (87.0%), excluding 11 cases of lymphocyte predominance type. The stain was better in B-5-fixed specimens than in formalin-fixed specimens, showing a dense deposit of reaction products at a paranuclear site and on the cell surface. The staining results were compared with those of Leu-M1 and found to be superior both qualitatively and quantitatively (detection rate of R-S cells: 87.0% versus 68.5% of Leu-M1). Granulocytes, rare epithelioid histiocytes, and some endothelial and/or erythrocytes also stained with anti-Lex. The stain had positive results in three cases of GS showing a diffuse cytoplasmic staining pattern. Of NHL, two of 29 peripheral T-cell lymphomas stained to show rare paranuclear deposits without cell surface staining. The stain had negative results in MH, ML, IRCS, HX, and RH. Of 45 reactive LN, minute subcapsular collections of Lewis X+, altered-appearing Langerhans'-like cells, were observed in all ten LN from human immunodeficiency virus (HIV)-associated persistent generalized lymphadenopathy (PGL). The stain had negative results in all other various reactive conditions of LN. In conclusion, Lewis X staining is useful as a marker for R-S cells in paraffin sections with staining results superior to those of Leu-M1. Lewis X staining also detects subcapsular clustering of altered-appearing Langerhans'-like cells in PGL, which has not been described previously and warrants additional study.
Cancer 1991 Mar 01
PMID:The Lewis X antigen. A new paraffin section marker for Reed-Sternberg cells. 170 18

Since multiple primary malignant tumors are rare in children, their presence can be a diagnostic and therapeutic problem. In this report, we present a six-year-old boy with Langerhans cell histiocytosis and Hodgkin's disease. On admission, the patient had lytic lesions and a periosteal reaction on the left trochanter major from which an open bone marrow biopsy was performed. The biopsy revealed Langerhans cell histiocytosis. Eight months later, the child presented with enlarged left cervical lymph nodes and the biopsy demonstrated Hodgkin's disease. Although there was an eight-month interval between the two histopathological diagnoses, the diffuse pulmonary parenchymal infiltration observed on the first admission, was later confirmed by an open-lung biopsy as Hodgkin's disease. The patient was said to have two concurrent lymphoreticular malignancies. To our knowledge, this is the youngest case reported with this association in the English language literature.
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PMID:Multiple primary malignancy: a report on Langerhans cell histiocytosis associated with Hodgkin's disease. 179 99

We describe an adult with progressive LCH who received oral etoposide as primary treatment. The response is documented and the strategical implications of this drug in Langerhans-cell histiocytosis discussed. Langerhans-cell histiocytosis (LCH) is the term recognized since 1987 for the group of diseases previously designated histiocytosis X. It is not now regarded as a malignant neoplastic process and there is some evidence to suggest that it is due to abnormal immunity, but cytotoxic drugs and steroids are still the mainstay of systemic treatment. Etoposide (VP16) is a semisynthetic epipodophyllotoxin derivative effective in the treatment of malignancies of the monocyte-macrophage lineage and used in resistant or relapsed childhood LCH. There are no previous reports of its use as firstline monotherapy in adults with LCH.
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PMID:Langerhans-cell histiocytosis--excellent response to etoposide. 179 74

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

Histiocytosis X (HX) is characterized morphologically by a proliferation of Langerhans' cells (LC), and most often has an indolent, chronic course. To determine whether a distinct clinicopathologic entity of malignant histiocytosis X exists, the authors examined tissues from 31 patients with HX and divided them into four categories. Group A (19 patients) was characterized morphologically by benign-appearing LC and had an indolent course. The male:female (M:F) ratio was 10:9, and the mean age was 21 years (range, 2 months to 60 years). The immunophenotype of this group was S-100+, vimentin+, LN-2+, LN-3+, lysozyme-, LCA-, Leu-M1-. Group B (three patients) had benign-appearing LC, yet had an aggressive clinical course. All patients were male, with a mean age of 47 years (range, 3 years to 72 years). Organs involved included the liver, spleen, heart, thymus, lung, kidney, and pancreas. The immunophenotype was the same as for Group A. Group C (two patients) had atypical and malignant appearing LC, yet a relatively benign clinical course. The ages were four and 65 years, with one female and one male patient. In both patients, the cells were S-100+, vimentin+, LN-2+, LN-3+, and LCA-. Group D (seven patients) was characterized by atypical and malignant-appearing LC and an aggressive clinical course. The mean age was 25 years (range, congenital to 54 years) with one female and six male patients. Organs involved were the thymus, lungs, spleen, liver, kidney, brain, heart, pancreas, stomach, and muscle. Birbeck granules were found in two patients, and the one patient on which fresh tissue was available was CD1+. The typical immunophenotype was S-100+, vimentin+, LN-2+, LN-3+, Leu-M1-, lysozyme-. The results of our study indicate that (1) a distinct clinical entity of malignant HX, characterized morphologically by malignant-appearing LC and clinically by male predominance, atypical organ involvement, and an aggressive clinical course, does exist; and (2) the morphologic appearance of the LC is an imperfect predictor of the clinical severity of HX.
Cancer 1991 Sep 01
PMID:Malignant histiocytosis X. A distinct clinicopathologic entity. 191 75

A retrospective analysis of 21 Saudi children with Histiocytosis-X were reviewed. 13 were males, 8 were females, with a ratio of 1.62:1. Five had unifocal disease while 16 were with multifocal disease. Bone involvement was seen in 19 patients (90.4%), and three patients had organ dysfunction. One patient had family history of Histiocytosis-X. Follow-up was from 1-10 years (Median 3 years). Three patients died, three had recurrences and eight patients had various disabilities (38%). Overall disease free survival was 84.2 percent.
Indian J Cancer 1991 Jun
PMID:Histiocytosis-X in Saudi children. 193 47

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.
Cancer 1991 Mar 15
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

Tissues from two patients with disseminated histiocytosis X (Letterer-Siwe disease) in which histiocytosis X cells exhibited histologic and cytologic features of malignancy were evaluated by in situ hybridization with the use of biotinylated nucleic acid probes to c-myc and H-ras oncogenes. Enhanced expression of these oncogenes was observed in mononucleated and multinucleated cells of histiocytosis X in the terminal proliferative phase but not in the early quiescent phase of Letterer-Siwe disease in both patients. Our findings indicate that deregulation of c-myc and H-ras in histiocytosis X are late events that likely confer a selective growth advantage to histiocytosis X cells.
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PMID:Enhanced expression of c-myc and H-ras oncogenes in Letterer-Siwe disease. A sequential study using colorimetric in situ hybridization. 225 22

A group of proliferative diseases of the epidermal Langerhans' cells are commonly referred to as Langerhans' cell histiocytosis (LCH). A small number of the patients with this disease face an unfavorable disease course despite chemotherapy and radiation therapy. In LCH patients with a poor prognosis, allogeneic bone marrow transplantation (BMT) could be the appropriate treatment with proven antiproliferative effects and may be able to repopulate the recipient with stem cell-derived Langerhans' cells from the donor or correct the presumed underlying immunodeficiency. An LCH was diagnosed in a 15-year-old boy with multiple osteolytic lesions, anemia, and diabetes insipidus centralis. Repeated flare-ups of the disease had necessitated several courses of conventional chemotherapy including cyclophosphamide (CY), prednisolone (P), 6-mercaptopurine (6-MP), vincristine (VCR), and additional local irradiation without stable remission. Three years after first being diagnosed with LCH the patient underwent high-dose chemotherapy-radiation therapy followed by allogeneic BMT from his human lymphocyte antigen (HLA)-identical brother. Currently, he is alive and well and has been disease-free for more than 41 months after BMT.
Cancer 1990 Jul 15
PMID:Allogeneic bone marrow transplantation for Langerhans' cell histiocytosis. 236 12


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