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

The work was undertaken owing to great difficulties in diagnosis and treatment of histiocytosis X (HX) in children, which occurs comparatively rarely and its clinical symptoms are of great variety. The analysed material included 45 cases of children with HX treated in one clinical department. A big number of analysed cases makes safe conclusions possible. The aim of this work was evaluation of therapeutic methods used in HX, on the basis of the results obtained from the HX treatment and with consideration of such prognostic factors as: the age of the child when the first pathological symptoms occurred , the extent of the pathological process and the localization of the lesions. The results of the research carried out on 23 children in whom the pathologic process concerned the skeletal system only were evaluated separately. Remaining 22 cases in which the lesions were noticeable not only in the skeletal system, but also in other organs and systems were divided in two groups: group 1 (11 children) included all the cases treated till 1969 by different methods with the use of different cytostatics; group 2 (11 children) included the cases observed by the author introducing a new method of treatment based on intensive therapy followed by the usage of Vinblastine or both Vinblastine and Prednisone. In the evaluation of the extent of the pathologic process and localization of lesions the following symptoms were taken into consideration: skeletal and skin lesions, enlargement of lymphatic nodes and liver, lesions of gums--i.e. movable teeth, lesions in ears, lungs, hematopoietic system, and fever. The presence of these symptoms is proved by the clinical, radiological and laboratory examinations. The age of the patient with the first symptoms of the disease was established through inquiry, the appearance at least one of the HX symptoms such as: protrusion of the soft tissue, skin lesions and discharge from the ears was considered as the beginning of the disease. In the group of children with lesions limited only to skeletal system, the age of the appearance of the first symptoms was between 1-12 years. The greatest morbidity was between the 4-th and the 8-th year of life (Fig. 1). The main methods of treatment were surgery and/or radiotherapy. Chemotherapy was used sporadically as supplementary treatment. In all cases response was good. (Tab. 1.). The generalized form of the disease is observed as a rule among the youngest children. In our own material of 20 cases out of all 22, the first symptoms of the disease appeared before 3-rd year of life was over. (Fig. 4.).(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Evaluation of the methods of treatment of histiocytosis X in children in relation to its clinical picture]. 31 11

Eosinophilic granuloma is a solitary form of histiocytosis X with a usually good prognosis. Sometimes spontaneous healing can even occur. The authors report a case of a 30-month-old boy who presented an eosinophilic granuloma of the lateral orbital wall. The correct diagnosis was obtained after computed tomographic scan followed by fine needle aspiration biopsy with histopathologic examination. The tumor increased rapidly, including a medial displacement of the globe and limitation of the eye movement. So chemotherapy (Vinblastine) with corticotherapy was performed. The clinical course was favorable, with no evidence of recurrence or systemic involvement. The originality of this case was the young age of the patient, the course and the efficacy of treatment. The authors emphasize the management of the disease.
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PMID:[Solitary eosinophilic granuloma of the external wall of the orbit]. 191 25

The brains of eight patient with Langerhans Cell Histiocytosis (LCH) were studied with magnetic Resonance Imaging (MRI). One scan was normal and a spectrum of abnormalities was seen in the others. Five patients had absence of the posterior pituitary bright spot, and four with evidence of pituitary dysfunction had a lesion in the region of the hypothalamus. An orbital extraconal mass was noted in one child with exopthalmus. This decreased in a follow-up study after Vinblastine therapy. The cerebellum was abnormal in two patients; the area of the dentate nuclei exhibited abnormal signal in one asymptomatic child and the MRI of another boy with a ten year history of progressive cerebellar dysfunction showed cerebral and cerebellar atrophy. Three of the five patients with deficiency of antidiuretic hormone (ADH) were studied both before and after the administration of intravenous Gadolinium-DTPA. In all three, after administration of the contrast agent, there was enhancement of involved areas.
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PMID:Brain MR in patients with Langerhans cell histiocytosis: findings and enhancement with Gd-DTPA. 239 58

A 4-year-old boy with skin symptoms resembling verruca plana juvenile was observed. The light- and electron-microscopic picture showed the typical features of histiocytosis X (Letterer-Siwe syndrome). Beside the skin manifestations some less dense areas in the skull bones were found by X-ray examination. Vinblastine and prednisolone treatment for one year resulted in complete remission.
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PMID:Histiocytosis X with unusual skin symptoms. 617 39

The authors are dealing with the therapy problems of disseminated Langerhans cell histiocytosis on the basis of seven patients. In the treatment the combination of Vinblastine and Prednisolone was usually applied. The therapy was successful in four cases. The most significant prognostic factors were found to be the presence of organ dysfunction (mainly hepatic and pulmonary), the bone involvement and immunological parameters, respectively. Real therapy goals are stressed.
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PMID:[Therapeutic problems in disseminated histiocytosis X]. 833 43

In a retrospective study involving 32 haematology/oncology departments in France, 348 cases of Langerhans' cell histiocytosis diagnosed between 1983 and 1993 were collated. The percentage of males was 56.4%. Median age at diagnosis was 30.2 months. The median follow up was 35.5 months. Initially, 108 patients (31%) had isolated unifocal or bifocal bone involvement, 67 (19%) had isolated multifocal bone involvement, 136 (39%) had soft tissue involvement without organ dysfunction, and 37 (11%) had organ dysfunction. Two thirds of the sites of involvement diagnosed throughout the course of the disease were present at diagnosis, while the remaining one third appeared during a relapse. Treatment was tailored to the individual patient and was extremely varied, hampering any comparison of regimens. Vinblastine with or without steroids was the most common regimen when systemic chemotherapy was used for the first episode (246/348). Twenty four of the 216 patients received VP 16 as first line treatment. Two patients with progressive multiorgan relapse, despite the use of several drugs, underwent bone marrow transplantation and are alive and disease free 60 and 22 months later. Altogether 21.9% of patients had sequelae, including diabetes insipidus in 17.5% of cases. The overall survival rate is 91.7% (confidence interval 90.7 to 95%) three years after diagnosis. In the univariate analysis, age less than 1 year, ear, nose, and throat, cutaneous, lymph node, liver, spleen, lung, marrow and intestinal involvement, male sex, progressive episodes, the absence of response, and partial responses, were associated with a poor vital prognosis. In a multivariate analysis of prognostic factors, poor early outcome emerged as the most important parameter, closely linked to other poor outcome features such as young age and organ dysfunction. It identified a small number of patients with a poor initial response to treatment, for whom intensive treatment should be assessed in a phase II trial.
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PMID:A multicentre retrospective survey of Langerhans' cell histiocytosis: 348 cases observed between 1983 and 1993. The French Langerhans' Cell Histiocytosis Study Group. 881 65

We make a retrospective evaluation of clinical and radiologic features, treatment, and outcome of Erdheim-Chester disease, a rare non-Langerhans cell histiocytosis. We report a case of Erdheim-Chester disease and review 60 cases from the literature. These cases are consider to have Erdheim-Chester disease when they have either typical bone radiographs (symmetrical long bones osteosclerosis) and/or histologic criteria disclosing histiocytic infiltration with distinctive immunohistochemical phenotype of the non-Langerhans cell histiocytes with positive staining for CD68 and negative staining for S-100 protein and CD1a. Our patient undergoes chemiotherapy according to the LCH-II stratification and therapy plan (Vinblastine, Etoposide and Prednisone) and thereafter receives Carboplatin and Etoposide, and Somatostatin. She is alive and clinically well 33 months after onset of symptoms and the lesions don't appear to progress at imaging examinations. In conclusion, Erdheim-Chester disease may be confused with Langerhans cell histiocytosis as it sometimes shares the same clinical (exophthalmos, diabetes insipidus) or radiologic (osteolytic lesions) findings. However, the characteristics radiological pattern of Erdheim-Chester disease together the immunohistochemical phenotype of hystiocytic infiltration supports the theory that Erdheim-Chester disease is a unique disease entity distinct.
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PMID:[Erdheim-Chester disease: a non-Langerhans cell histiocytosis. A clinical-case and review of the literature]. 1534 69

Langerhans cell histiocytosis (LCH) is a disorder with unclear etiology and pathogenesis, which is characterized by abnormal clonal proliferation and accumulation of langerhans cells at various tissue and organs. A total of 217 patients with LCH were evaluated retrospectively for clinicopathological features, laboratory findings, treatment modalities, long-term outcome, and factors affecting the outcome. Median age at the time of diagnosis was 3.5 years and male/female ratio was 1.8. The most common complaint at presentation was a bone lesion-related symptom. Fifty percent of the patients younger than 2 years had organ dysfunction (OD). Treatment consisted of surgery, chemotherapy, and radiotherapy alone or in combination. Vinblastine with or without prednisolone was the most common used chemotherapy regimen. Overall (OS) and event-free survival (EFS) rates were 84% and 51.5%, respectively, at an 8-year median follow-up time. Overall survival was significantly lower in patients younger than 2 years of age and patients with OD. The age at diagnosis, pulmonary, liver, or hematological involvement, and elevated acute-phase reactants were found to have a statistically significant effect on the OS or EFS rates.
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PMID:Langerhans cell histiocytosis: retrospective analysis of 217 cases in a single center. 1856 42