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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 gastrointestinal tract may be involved in Langerhans' cell histiocytosis, either as part of a generalized disease or as a separate primary entity. Three patients with gastrointestinal involvement are described. One patient presented with a history of prolonged vomiting and diarrhoea with blood and mucus. In this case the diagnosis was based on colonic biopsies. Gastrointestinal tract involvement and its investigation in the diagnosis of Langerhans' cell histiocytosis are discussed.
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PMID:Gastrointestinal involvement in Langerhans' cell histiocytosis (Histiocytosis X): a clinical report of three cases. 217 34

A chronic diarrhea accompanied by vomiting and weight loss was the major symptom in a one-year-old infant during a fulminant course of histiocytosis X. The diagnosis was suggested by the radiologic evidence of alternating dilated and stenotic segments in the small and large bowel as well by a massive intestinal protein loss and the presence of histiocytes in the mucosa and submucosa of the rectum. The histological picture of enlarged mesenteric lymph nodes, obtained during a diagnostic laparotomy, confirmed the diagnosis.
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PMID:[Chronic diarrhea as the main symptom of histiocytosis X]. 278 13

The authors report a case of histiocytosis X which presented with neurological manifestations in a 20 year old man: episodes of headache with vomiting, followed, three months later, by the development of paralysis of the right 6th and 7th cranial nerves associated with nystagmus. The CT scan revealed a tumour-like lesion on the floor of the IVth ventricle with a long axis of 18 mm. The chest x-ray revealed diffuse nodular opacities in the pleural and apical regions with features suggestive of histiocytosis X. The diagnosis was confirmed by surgical biopsy of the typical pulmonary nodules which were rich in histiocytes with X bodies on electron microscopy. The neurological signs disappeared after one month of treatment with Prednisone (1 mg/kg/day) and Vincaleukoblastine (10 mg/week). By the 3rd month, the pulmonary lesions were reduced and the intra-ventricular formation had regressed by 40%. In the authors' series of 29 cases of confirmed histiocytosis X in adults, the present case is the only one with a clinical neurological presentation, apart from 3 cases of diabetes insipidus. A review of the literature confirms the rarity of this type of presentation. The suggestion of the diagnosis by the chest x-ray appearance enabled a dangerous neuro-surgical operation to be avoided.
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PMID:[Cerebral and pulmonary histiocytosis X. Neurologic manifestations disclosing a pseudotumoral formation on the floor of the 4th ventricle]. 633 82

An 11-year-old black boy complained of intermittent occipital headaches with nausea and projectile vomiting. Previous skin and lung biopsy specimens were interpreted as histiocytosis X. Cranial computed tomographic scanning disclosed a mass lesion in the region of the choroid plexus of the left lateral ventricle. This was surgically removed but proved nondiagnostic despite extensive histologic examination. An ophthalmologic evaluation showed discrete, elevated, yellow-white choroidal tumors in both maculas. The ophthalmoscopic appearance, as well as ultrasonography and computed tomography, led to the diagnosis of choroidal osteomas.
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PMID:Bilateral choroidal osteomas associated with fatal systemic illness. 697 70

An 8-year-old boy developed vomiting and severe headache following minor head trauma. A CT scan of the head demonstrated a lytic lesion of the skull and adjacent epidural hematoma. Surgical evacuation and removal of the skull lesion and hematoma were carried out, and pathologic evaluation resulted in a diagnosis of Langerhans' cell histiocytosis (LCH). Epidural involvement of Langerhans' cell histiocytosis is very rare, and we report the first case of LCH presenting as an intracranial epidural hematoma.
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PMID:Langerhans' cell histiocytosis presenting with an intracranial epidural hematoma. 1083 96

A case of disseminated Langerhans cell histiocytosis with resistant central nervous system (CNS) disease in an adolescent is described. The child presented with visible cranial lesions, emesis, headaches, and short-term memory loss. Diagnostic evaluation revealed multiple osseous lesions in the cranium, ribs, vertebral bodies, and pelvis. The clinical course with complications and response to each therapy are sequentially reviewed. Remission, as evidenced clinically and by magnetic resonance imaging, was ultimately accomplished with 2-chlorodeoxyadenosine (2-CDA). The full course of 2-CDA was not tolerated due to bone marrow suppression. CNS histiocytosis is known to be resistant to therapy. Earlier introduction of 2-CDA for CNS disease might offer more successful treatment with less toxicity than seen in patient.
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PMID:Langerhans cell histiocytosis: central nervous system involvement treated successfully with 2-chlorodeoxyadenosine. 1129 88

Benign intracranial hypertension is known to be associated with obesity, endocrine abnormalities, various medications, and cerebral venous sinus thrombosis. We report a patient presenting with headaches and vomiting attributed to benign intracranial hypertension. The diagnostic work-up revealed Langerhans' cell histiocytosis of the occipital bone. There was no evidence for cerebral vein thrombosis by cranial computed tomography scan, Doppler ultrasonography, planar and single photon emission computed tomography technetium 99m-labelled red blood cell scintigraphy, and magnetic resonance angiography. Excision of the occipital bone lesion and a short course of acetazolamide and prednisone were curative. We hypothesize that cytokines secreted by the tumor were responsible for the development of intracranial hypertension.
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PMID:Langerhans' cell histiocytosis presenting as intracranial hypertension. 1141 6

Gastrointestinal tract (GIT) involvement in Langerhans cell histiocytosis (LCH) is not commonly described. We present two children presenting with GIT involvement with LCH, one successfully treated on standard protocol and other being treated on a protocol for relapsed disease. A review of literature showed almost 95% children were less than 2 years of age and 62% were females. Vomiting, abdominal pain, constipation, intractable diarrhea, malabsorption, bloody stools, protein-losing enteropathy, and even intestinal perforation are some of the reported symptoms. More than 50% patients died within 18 months from diagnosis.
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PMID:Langerhans cell histiocytosis with digestive tract involvement. 2053 29

Langerhans cell histiocytosis (LCH) with involvement of the gastrointestinal tract is rare and typically identified in patients with systemic disease. We describe a 16-month-old girl who initially presented with bilious vomiting, failure to thrive and a rash. An upper gastrointestinal (GI) examination revealed loss of normal mucosal fold pattern and luminal narrowing within the duodenum, prompting endoscopic biopsy. Langerhans cell histiocytosis of the digestive tract was confirmed by histopathology. A skeletal survey and skin biopsy identified other systemic lesions. Although uncommon, it is important to consider LCH in the differential diagnosis for gastrointestinal symptoms of unclear origin, especially when seen with concurrent rash. Findings of gastrointestinal involvement on upper GI examination include loss of normal mucosal fold pattern and luminal narrowing in the few published case reports.
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PMID:Langerhans cell histiocytosis of the digestive tract identified on an upper gastrointestinal examination. 2688 14

Eosinophilic granuloma (EG) represents a local form of Langerhans cell histiocytosis that occurs mostly in children. It usually presents with a gradually enlarging painless skull mass, and rarely presents a rapid clinical deterioration. This 7-year-old boy who was diagnosed with EG, based on a magnetic resonance imaging scan, after presenting with a painless right parietal swelling of 7-week duration. Three weeks prior his scheduled surgery, he presented to the emergency department with a 2-day history of sudden increased of the subcutaneous swelling associated with a headache, vomiting, and decreased the level of consciousness; there was no history of trauma. Brain computed tomography revealed a right parietal bone defect with large subgaleal and extradural hematoma. He underwent emergent surgical excision of the skull lesion and evacuation of the hematoma. Histopathological examination confirmed the diagnosis of EG. We aim to raise the awareness of physicians of this rare spontaneous hemorrhagic complication of EG and review the literature.
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PMID:Spontaneous extradural hemorrhage due to Langerhans cell histiocytosis of the skull in a child: A rare presentation. 2719 34


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