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)

A 42-year-old woman with a 6-year history of diabetes insipidus and progressive hypersomnolence presented with visual loss. Neuroimaging showed infiltration in the hypothalamus, the optic nerve, and the chiasm, as well as multiple lesions in other areas of the brain parenchyma. Biopsy showed Langerhans cell histiocytosis. This is an unusual presentation of Langerhans cell histiocytosis, involving the visual pathways without manifestations outside of the central nervous system. The differential diagnosis and the magnetic resonance imaging findings will be discussed.
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PMID:Visual loss with Langerhans cell histiocytosis: multifocal central nervous system involvement. 1009 49

A 42-yr-old woman was referred to our hospital because of multiple small nodules in a chest radiograph. She had no symptoms such as dyspnea, cough or sputum. A chest CT revealed many centrilobular small nodules and thin-walled cysts with predominance in the peripheral area of the lungs. The specimens obtained by thoracoscopic surgery showed granulomas with scattered eosinophils and numerous Langerhans' cells. The Langerhans' cells were positive for both S-100 protein and CD1a. These findings are compatible with pulmonary Langerhans' cell histiocytosis (LCH). Since the granulomas showed no fibrotic changes, the LCH may have been in its early stages. However, there were clusters of lymphocytes and macrophages around the terminal and respiratory bronchioles, and cystic lesions without cellular infiltrates, in the specimens. The former histologic findings suggested respiratory bronchiolitis causing interstitial lung disease and the latter are indistinguishable from centrilobular emphysema. Therefore, these smoking-related diseases may have been superimposed on the LCH in this patient.
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PMID:[A case of pulmonary Langerhans' cell histiocytosis]. 1453 8

A 42-year-old woman who had a cigarette index of 420 had many cavitary lesions predominantly in the upper areas of both lungs. The lesions were detected on a health examination using CT. Analysis with bronchofiberscopy showed increased CD1a positive cells in bronchoalveolar fluid (BALF). Histological examination by video-associated lung biopsy demonstrated that both S-100 and vimentin-positive cells were present in the peribronchial fibrotic lesions. From these data, this disease was diagnosed as pulmonary Langerhans cell histiocytosis (PLCH). Three months after the cessation of smoking, the cavity lesions disappeared. As the image findings improved, CD1a positive cells in BALF decreased (4.9% --> 1.8%) and the CD4/CD8 ratio in BALF increased (1.66 --> 6.16). So far, there is no report describing the time course of both CD1a positive cells and CD4/CD8 ratio in BALF after cessation of smoking in PLCH. These findings attract our interest on the PLCH.
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PMID:[A case of pulmonary Langerhans cell histiocytosis discovered by CT mass screening and followed by bronchoalveolar lavage]. 1714 89

Eosinophilic granuloma is the localized form of Langerhans' cell histiocytosis. There are several reports of magnetic resonance (MR) imaging of eosinophilic granuloma of the skull, however there are few reports about dural enhancement. We report an operative case of eosinophilic granuloma of the skull with dural invasion. A 42-year old man was admitted to our hospital. He was neurologically intact and there were no other osseous or soft tissue lesions. CT showed an osteolytic lesion in the left parietal bone. MR images showed the lesion as isointense on T1-weighted, and high intense on T2-weighted images. T1-weighted images with Gd-DTPA demonstrated the mass which was enhanced with dural surface and subgaleal tissue. The angiogram demonstrated a tumor stain fed by the left occipital artery. Bone scintigraphy demonstrated a solitary lesion showing peripheral uptake with a central defect. The patient underwent craniectomy with removal of the dura and a subgaleal lesion. Histological examination revealed characteristic eosinophilic granuloma with dural invasion. No recurrence of the lesion was demonstrated 6 months after surgery.
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PMID:[Operative case of eosinophilic granuloma of the skull with dural invasion]. 1834 Oct 13

Langerhans cell histiocytosis is a rare disorder, with resultant bilateral sensorineural hearing loss unreported in adults. A 42-year-old man presented with 4 months of right-sided tinnitus and hearing loss treated initially as otitis media with effusion. He re-presented 5 months later with progressive bilateral hearing loss--sensorineural (>100 dB) on pure tone audiogram. CT showed bilateral petrous temporal bone and calvarial lesions. Biopsy confirmed diagnosis of Langerhans cell histiocytosis and chemotherapy was started. Though uncommon, Langerhans cell histiocytosis should be considered among the differentials of persistent otological symptoms, as its progressive nature can cause bilateral irreversible sensorineural hearing loss.
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PMID:A case of bilateral sensorineural hearing loss from Langerhans cell histiocytosis. 2634 Nov 62

Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed to show the posterior pituitary, which is a typical finding in patients with central diabetes insipidus. This condition was confirmed by a water deprivation test, and the patient was also found to have type 2 diabetes mellitus. Computed tomographic scanning of the lungs revealed multiple, irregularly shaped cystic lesions and small nodules bilaterally, with sparing of the costophrenic angles. Lung biopsy through video-assisted thoracoscopic surgery revealed pulmonary Langerhans cell histiocytosis. On a follow-up visit, only 1 year after the patient had quit smoking, clinical and radiological improvement was significant. Here, we report an uncommon case of pulmonary Langerhans cell histiocytosis that simultaneously presented with diabetes insipidus and diabetes mellitus.
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PMID:Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report. 2650 47