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

Histiocytosis X, a clinical entity which was not defined and classified exactly so far, is manifested by a varied range of clinical manifestations. In rare instances the affection of the orbit is the only and first symptom of the disease. The authors present an account of three children. In one isolated affection of the orbit with a non-inflammatory swelling of the external portion of the upper eyelid was the first symptom of the disease. In the other two patients with the acute multifocal form of histiocytosis X exophthalmos developed only 3 and 7 months resp. after establishment of the diagnosis. In the discussion of symptoms, the prognosis and treatment the authors are inclined to believe that at present the prognosis of multifocal form of the disease is not always adverse. In the frequently difficult differential diagnosis of orbital lesions histiocytosis X should be considered as one of the possible causes of affections of the orbit in children.
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PMID:[Histiocytosis X and the orbit in children]. 152 90

Histiocytic tumors of the orbit comprise an unusual group of lesions characterized by the infiltration of the orbital tissues by xanthomatous cells. Recently, there have been a number of new observations regarding the various histiocytic tumors that can affect the orbit. The condition previously referred to as histiocytosis X is believed to represent a proliferation of Langerhans cells and the term Langerhans cell histiocytosis is often used instead of histiocytosis X. Juvenile xanthogranuloma has been demonstrated to affect the orbit without involving the skin or the iris. The Erdheim-Chester disease is a condition of adults characterized by infiltration of bone, retroperitoneum, heart, lungs and other tissues by xanthoma cells. This condition has recently been recognized to produce a classic ophthalmological picture of bilateral xanthelasmas and bilateral proptosis. The authors review their personal experience with several patients with histiocytic tumors of the orbit and stress the clinical spectrum of these conditions.
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PMID:Clinical spectrum of histiocytic tumors of the orbit. 208 89

Diabetes insipidus is a well-recognized complication of Langerhans-cell histiocytosis (histiocytosis X), but its frequency and natural history are not well defined. Of 52 children with histiocytosis whom we studied, 12 (23 percent) had diabetes insipidus. Only two children had diabetes insipidus at presentation with histiocytosis, but the cumulative risk that it would develop during the first four years after the presentation and diagnosis of histiocytosis was found to be 42 percent. Diabetes insipidus occurred most often among children with multisystem disease and those with proptosis. To determine the natural history of diabetes insipidus in children with histiocytosis, we measured the response of urinary arginine vasopressin to water deprivation every six months in 21 children who did not have diabetes insipidus and who had had histiocytosis for less than four years. Five of the 21 children (24 percent) had subnormal responses during the initial test. One subsequently had spontaneous improvement in the functioning of the posterior pituitary, and diabetes insipidus subsequently developed in two, as it did in one of the children who initially had normal function of the posterior pituitary. Two of the children received irradiation to the pituitary within two to four weeks after diabetes insipidus developed, but they had no improvement in pituitary function. However, diabetes insipidus improved transiently during prednisolone therapy in one of these children and improved permanently after etoposide therapy in another child. We conclude that prospective study with the use of a simple water-deprivation test will allow partial defects of posterior-pituitary function to be detected in patients with histiocytosis and will permit a more appropriate evaluation of the effects of therapeutic intervention.
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PMID:The frequency and natural history of diabetes insipidus in children with Langerhans-cell histiocytosis. 279 79

Of 76 children with histiocytosis X 18 had orbital involvement, and four developed additional neuro-ophthalmic complications. No instance of intraocular involvement was detected. Among those patients with ophthalmic involvement the main problems were bilateral or unilateral proptosis, ptosis, papilloedema, optic atrophy, and seventh nerve palsy. Only one patient developed a severe visual defect. Management of the ophthalmological complications depends not only on the extent of the orbital disease but also on the degree of systemic involvement. Overall management by a paediatric oncologist is mandatory.
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PMID:Histiocytosis X: an ophthalmological review. 387 Nov 58

The authors experienced a case of histiocytosis X with a large intracranial mass resulting in a convulsive seizure. The patient showed left exophthalmos and a skin rash one year and two months after birth. Histiocytosis X was diagnosed from a skin biopsy, and predonine, endoxan and vincristine were administered. The rash disappeared, but the exophthalmos remained. At the age of two years and nine months, punched-out lesions appeared in the skull and 4,000 rads of radiation was applied. Thereafter, the exopthalmos persisted but there was no particular problem in the course. However, a convulsive seizure with fever suddenly appeared at nine years and ten months of age and the patient was hospitalized. At the time of admission, the general condition was good and there were no abnormalities in neurological tests. In neuroradiological examinations, a calcified and poorly vascularized mass 8 cm in maximum diameter was found to occupy the left middle cranial fossa. Chondrosarcoma was strongly suspected from these findings, but there was also symmetrical thickening of bone cortex in the peripheries of the long bones of the extremeties which appeared to be the recovery process from bone destruction caused by histiocytosis X. Therefore, the formation of an intracranial mass by histiocytosis X was diagnosed and surgery was performed. When left osteoplastic fronto-temporal craniotomy was performed, the mass was found to be raising the temporal lobe and it could be easily separated from the surrounding tissue. However, these was firm adherence to dura mater of the middle cranial fossa (especially that of the superior orbital fissure). Histologically, there were many cells with small nuclei, no polymorphism, abundant and clear cytoplasm which were darkly stained and slightly atypic. These findings matched those for histiocytosis X. Cases of histiocytosis X rarely show symptoms of the central nervous system or infiltration of the central nervous system. Only 31 such cases were seen in the literature investigated by the authors. Neurological symptoms include pyramidal symptoms such as hemiparesis and impairment of the cranial nerves, particularly paresis of the optic, trigeminal, facial and acoustic nerves. Convulsive seizures were seen in only five cases including the one reported here. It is also rare for intracranial masses to be formed in cases of histiocytosis X and only six cases, including the authors', have been found with masses of a maximum diameter of more than 5 cm.
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PMID:[Case of histiocytosis X with a large intracranial mass]. 660 36

The ophthalmological changes in two patients with Erdheim-Chester disease are described. These consist of exophthalmos, ophthalmoplegia, xanthelasma, optic disc swelling, blindness due to optic atrophy, retinal striae, and bilateral enhancing orbital masses on CT scan. The clinical and histopathologic findings of Erdheim-Chester disease are reviewed and the manifestations in two patients with orbital change are presented. This is believed to be the first report describing the ophthalmological manifestations of Erdheim-Chester disease. Our observations support the view that Erdheim-Chester disease is unrelated to the histiocytosis X group.
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PMID:Orbital manifestations of Erdheim-Chester disease. 667 81

We made a retrospective evaluation of clinical and radiologic features, treatment, and outcome of Erdheim-Chester disease, a rare non-Langerhans cell histiocytosis. We had 7 patients coming from 3 French teaching hospitals and reviewed 52 cases from the literature. These cases were considered to have Erdheim-Chester disease when they had either typical bone radiographs (symmetrical long bones osteosclerosis) and/or histologic criteria disclosing histiocytic infiltration without features for Langerhans cell histiocytosis (no S-100 protein, no intracytoplasmic Birbeck granules). Ages at diagnosis ranged from 7 to 84 years (mean +/- SD = 53 +/- 14 yr) with a male/female ratio of 33/26. Bone pain was the most frequent clinical sign (28/59), mostly located in the lower limbs. Exophthalmos and diabetes insipidus were found in respectively 16/59 and 17/59 patients. General symptoms (fever, weight loss) and "xanthomas" (mainly located on the eyelids) were present in 11/59 patients. Retroperitoneal involvement was found in 17/59 patients. Skeletal X-ray showed typical osteosclerosis of the diaphysis of the long bones in 45/59 patients. Bone radiographs showed osteolytic lesions of the flat bones (skull, ribs) in 8 patients. Histologic diagnosis was performed after a bone biopsy (28 patients), a retroorbital biopsy (9 patients), and/or a biopsy of the retroperitoneal infiltration or the kidney (11 patients). Six of our 7 patients but only 5 of 52 patients from the literature had the complete histologic criteria, disclosing no Birbeck granules or S-100 immunostaining. In other cases, histologic results usually described a xanthogranulomatous infiltration by foamy histiocytes nested in fibrosis. Treatment was corticotherapy (20/59), chemotherapy (8/59), radiotherapy (6/59), surgery (3/59) and immunotherapy (1 patient). Twenty-two patients died after a mean follow-up of 32 +/- 30 mo (range, 3-120 mo). 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, it also appears to have distinctive features. Patients are older and have a worse prognosis than those with Langerhans cell histiocytosis, and the diagnosis relies on the association of specific radiologic and histologic findings.
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PMID:Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases. 1169 22

This study evaluates the permanent disabilities in children treated for Langerhans cell histiocytosis (LCH). From January 1983 to December 1993, 50 patients with newly diagnosed biopsy proven LCH were seen at the Regional Cancer Centre, Trivandrum, India. Disease pattern, treatment, survival, and disabilities of the patients were studied. Patients with localized disease had surgery, irradiation, or steroids. Patients with disseminated disease had combination chemotherapy. Follow-up ranged from 36 to 156 months (median follow-up 85 months). Twelve of the 41 surviving patients (29.2%) had one or more disabilities. Growth retardation was seen in 8 patients, diabetes insipidus in 7, loss of teeth in 6, and mandibular defect, chronic aural discharge, partial hearing loss, facial palsy, and proptosis in 2 each. In short, a significant proportion of survivors of LCH had sequelae, which affected their quality of life. More intensive chemotherapy at the beginning might be helpful in reducing the disabilities.
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PMID:Permanent disabilities in childhood survivors of Langerhans cell histiocytosis. 1091 47

Erdheim-Chester disease (ECD) is a rare multisystem histiocytosis syndrome of unknown cause that usually affects adults. Histiocytic infiltration of multiple end organs produces bone pain, xanthelasma and xanthoma, exophthalmos, diabetes insipidus, and interstitial lung disease. Differential diagnosis includes Langerhans cell histiocytosis, metabolic disorders, malignancy, and sarcoidosis. ECD can be diagnosed using a combination of clinical and histopathologic findings. Sites of involvement include lung, bone, skin, retroorbital tissue, central nervous system, pituitary gland, retroperitoneum, and pericardium. Symmetrical long bone pain with associated osteosclerotic lesions, xanthomas around the eyelids, exophthalmos, and/or diabetes insipidus suggest ECD. Approximately 35% of patients have associated lung involvement, characterized by interstitial accumulations of histiocytic cells and fibrosis in a predominantly perilymphangitic and subpleural pattern. This pattern distinguishes ECD from other histiocytic disorders involving the lung. The diagnosis is confirmed by tissue biopsies that contain histiocytes with non-Langerhans cell features. In general, the clinical course of patients with this disease varies, and the prognosis can be poor despite treatment. Clinical trials for treatment of ECD have not been conducted and treatment is based on anecdotal experience.
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PMID:Erdheim-Chester disease: a rare multisystem histiocytic disorder associated with interstitial lung disease. 1120 82

Hand-Schuller-Christian disease is a multifocal variant of eosinophilic granuloma, characterised by the classical triad of bony lesions, exophthalmos and diabetes insipidus. This case relates recurrent Langerhans' cell histiocytosis lesions presented as destruction of periodontal support associated with diabetes in a 10-year-old patient. Medical history suggests that the case represents a case of Hand-Schuller Christian disease.
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PMID:Langerhans cell histiocytosis: recurrent lesions affecting mandible in a 10-year-old patient. 1204 86


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