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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 of the female genital tract is a rare disease. A clinical diagnosis is impossible to establish because no typical lesions are found. Immunohistochemistry of bioptic samples is the easier technique to obtain the correct diagnosis. We present a case of a 19-year-old woman with Langerhans cell histiocytosis of mandible and maxilla with subsequent cervical and vulvar histiocytosis.
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PMID:Langerhans cell histiocytosis of vulva and cervix in a 19-year-old woman. 925 59

Cervical lymphadenopathies are widespread diseases in otorhinolaryngology. There are many differential diagnoses which have to be considered when swelling persists. To gain further information it is advisable to perform sonographically controlled fine needle aspiration biopsy (FNP). If there is no reliable result, it is necessary to remove a lymph node. We report on the rare case of isolated histiocytosis X in lymph nodes. A 63-year-old male had noticed a swelling of the right side of the neck about 6 weeks previously. The preliminary examinations (FNP/blood examinations) revealed no pathological findings. Only the histological examination delivered the diagnosis of histiocytosis X. Systemic manifestation was excluded by staging examinations. No further therapy was necessary after lymph node extirpation. The report demonstrates how to diagnose and treat this rare disease.
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PMID:[Isolated histiocytosis X as rare differential diagnosis of indistinguishable neck lymph node swelling. A case report]. 941 55

Langerhans cell granulomatosis, once called histiocytosis X, is a rare disease. A case of multifocal Langerhans cell granulomatosis in the bone, lymph nodes, skin and lungs of an 18-year-old man is described. Head CT and MRI showed a soft tissue mass of the left temporal bone. Lymph node and skin biopsies substantiated a diagnosis of Langerhans cell granulomatosis. A High resolution CT scan of the lung revealed a small cystic lesion, and bronchoalveolar lavage (BAL) showed an increased number of S-100 positive cells. Steroid therapy resulted in complete resolution, and no S-100 positive cells were obtained in the follow-up BAL study.
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PMID:[A case of multifocal Langerhans cell granulomatosis: a BAL follow up study]. 956 87

Histiocytosis X (HX) is a rare disease. There are many questions and debates in its etiology, pathology, clinical type and therapy. To enhance the recognition of otolaryngologists in HX of the ear and temporal bone and the level of diagnosis and therapy, 30 years' results of 21 patients with HX of the ear and temporal bone proved by histiopathology were reviewed, 18 patients were cured (85%), 6 cases were residual of diabete insipidus and dwarfism (28%), 3 cases died (14%). The clinical classification system should be based on the extention of diseases. Surgical curettage, radiation therapy are mainstays of treatment; chemotherapy should be used in patients with aggressive and disseminate diseases and etiology of histiocytosis should be studied on immune system that potentially aid in therapy.
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PMID:[Histiocytosis X of the temporal bone]. 964 Jun 97

Langerhans' cell histiocytosis (LCH), previously known as histiocytosis X, is a rare disease. It is characterized by the accumulation and proliferation of histiocytes, eosinophils and Langerhans' cells with Birbeck granules detected by electron microscopy. It involves single organs or systems or can present as a multisystem disease. The clinical presentation may vary widely, ranging from benign self-limiting types with spontaneous regression to slowly-progressive malignant disease. We report five cases of LCH with the same histopathologic basis but different outcome.
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PMID:[Langerhans' cell histiocytosis: various manifestations with the same histopathologic base]. 1246 53

The Langerhans cell histiocytosis is a rare disease with a clear preference of infancy and an incidence of 0.2 to 1.0/100,000 children occurring worldwide. In 60 to 70% of the cases the first manifestation is before the second year of age. The disease prefers the male sex in a male to female relation of 2:1. Occurrence in adult age is very rare. Up to 10% of the cases are congenital. Both the etiology and the pathogenesis of the disease are unknown. The clinical presentation is extremely variable and covers a wide spectrum from localized involvement with a favorable prognosis to disseminated, malignant and foudroyant course. We report on ten patients, six infants and children and four adolescents and adults. We point out the very variable morphologic manifestations, the different disease course and the therapeutic options.
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PMID:[Clinical course and therapy of Langerhans cell histiocytosis in children and adults]. 1259 Mar 10

Langerhans cell histiocytosis is a rare disease in children. However, its ability to present in many ways, to mimic other conditions, and to manifest itself in many organs makes it a fascinating disease for radiologists. This article reviews the history of the disease, the features that are most useful in determining prognosis, and the various radiological findings seen in paediatric patients.
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PMID:Paediatric manifestations of Langerhans cell histiocytosis: a review of the clinical and radiological findings. 1266 47

Langerhans cell histiocytosis is a rare disease with an unknown etiology and poorly understood pathogenesis. Immunologic, viral, and proliferative clonality causes have all been considered. To determine whether Langerhans cell histiocytosis and its two main subgroups, single-system and multisystem disease, are associated with HLA-A, -B, -Cw, or -DR alleles, a total of 84 patients <15 y of age at the time of diagnosis and of Nordic origin were analyzed, 82 for HLA class I and 76 for HLA class II. Stratification of the patients into two subgroups, single-system disease (skin only, and monostotic and polyostotic disease) and multisystem disease with or without organ dysfunction, showed that patients with single-system disease (17 of 45, 38%) more often (p = 0.00018 and, after correction, p = 0.029) had the phenotype HLA-DRB1*03 compared with patients with multisystem disease (1 of 31, 3%). In the patients with multisystem disease a nonsignificant reduction of the frequency of this phenotype was seen compared with controls (p = 0.02, uncorrected). In 14 of the patients with single-system disease, but none with multisystem disease, the deduced haplotype HLA-A*01, B*08, DRB1*03 was found. High-resolution typing, performed in nine patients, revealed that all had the HLA-A*0101, B*0801, DRB1*0301, DQB1*0201 alleles. Our findings suggest an immunogenetic heterogeneity in the two clinical entities of Langerhans cell histiocytosis and indicate that HLA-DRB1*03 may play a protective role against developing multisystem disease. Further studies to confirm these findings are desired.
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PMID:Immunogenetic heterogeneity in single-system and multisystem langerhans cell histiocytosis. 1270 Mar 69

Pituitary-hypothalamic axis Langerhans cell histiocytosis is an uncommon entity. It is a rare disease in adults. The CT and MR study provides us the best anatomo-topographic evaluation and determine the precise size of the lesion which are necessary to the treatment. We report a case of hypothalamic involvement by Langerhans cell histiocytosis accompanied by lesions in bone affecting a 31-year-old woman. The clinical, histiotological and CT/MR findings of histiocytosis X are described in this article.
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PMID:[MRI findings in pituitary-hypothalamic axis Langerhans cell histiocytosis]. 1284 12

Eosinophilic granuloma (EG) is a rare disease but is more common in adults than children. It's often self-limiting. Spinal involvement is rare. It is the localized and most benign form of Langerhans' cell histiocytosis (previously known as histiocytosis X), characterised by lytic lesions in one or more bones. Spontaneous resolution of vertebral body lesions is very rare. In this case, the patient had one EG in a cervical vertebra and a similar lesion in a lumbar vertebra. This case is important because it featured a symptomatic lesion in the cervical spine accompanied by an asymptomatic lesion in a lumbar vertebra. We treated the cervical lesion by surgical fusion and followed the lumbar lesion up conservatively, with the patient in a corset. After 8 years of follow-up, control MRI showed that the lumbar lesion had spontaneously resolved.
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PMID:Spontaneous resolution of lumbar vertebral eosinophilic granuloma. 1496 50


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