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 28-year-old woman was admitted to our hospital with the chief complaint of progressive gingival swelling with easy bleeding and loosening of teeth for about two years. The liver and spleen were not enlarged and there were no lymphadenopathy. The hemogram was normal. The skull X-ray showed floating teeth. CT scan of face showed destructive bony lesions over maxilla and left mandible with adjacent soft tissue swelling. Pathologic examination of the gingiva revealed that the oral mucosal tissue was heavily infiltrated with histiocyte-like Langerhans' cells. The Langerhans' cells showed positive immunostain for S-100 protein. Under electron microscope, ultrastructure of the Langerhans' cells revealed typical intracytoplasmic tennnis racquet-shaped structure with a central zipper-like striation (Birbeck's granules). Langerhans' cell histiocytosis was diagnosed. She received rdiotherapy with a total dose of 1,000 cGy in fractions and oral chemotherapeutic drugs. Painful gingival swelling subsided gradually. She was followed at our OPD for the past 10 months and there was no evidence of local recurrence. Langerhans' cell histiocytosis is a rare disease. The relevant literature about its distinct pathologic features, clinical course and treatment is reviewed.
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PMID:Langerhans' cell histiocytosis (histiocytosis-X) in a women with typical ultrastructure of Birbeck's granule: a case report. 899 34

The spectrum of histopathologic changes in four cases of chronic recurrent multifocal osteomyelitis encountered in our orthopedic outpatient clinic in the past 3 years was studied in conjunction with clinical and radiologic findings. All presented with pain with or without swelling in the affected region. Radiographically, the appearance of the lesions varied from a mixed picture of bone lysis and sclerosis with expansion to sclerosis alone to bone collapse. Bone scintigraphy demonstrated asymptomatic and separate foci of activity in all cases. Prior to biopsy, the clinical and radiologic differential diagnoses included Ewing's sarcoma, metastatic neuroblastoma, hematolymphoid malignancy, Langerhans cell histiocytosis and chronic infection, notably tuberculosis. The spectrum of histopathologic changes ranged from acute (acute inflammatory infiltration, active bone resorption and necrosis, reactive bone formation) to subacute (predominantly lymphocytic and plasma cell infiltration) to chronic inflammation (fibroblastic organization and bony sclerosis). Histologic changes correlated poorly with clinical features, but relatively well with radiologic findings. Lesional excision was performed in one case, cortical saucerization in another, while the final two cases received supportive treatment. All remained well 18-21 months post-therapy. Chronic recurrent multifocal osteomyelitis is a great clinical and radiologic mimic, which merits recognition by the pathologist. Awareness of the spectrum of histologic features encountered enables a correct diagnosis to be made in the appropriate clinical setting. The patient can thus be reassured of a favorable prognosis.
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PMID:Chronic recurrent multifocal osteomyelitis: a great clinical and radiologic mimic in need of recognition by the pathologist. 1023 Jun 89

A case of intraosseous xanthoma in a patient with a normal lipid profile is reported. Hyperlipidemia is present in most patients with xanthomas. Intraosseous xanthomas are rare, particularly in normolipidemic patients, in whom the presenting symptom is pain without skin lesions. A lytic lesion with a rim of sclerosis is seen on radiographs. Histology shows foam cells, giant cells, and fibrosis. Intraosseous xanthoma is a benign tumor, and other diagnoses must be ruled out (histiocytosis X, Erdheim Chester disease, clear cell carcinoma metastasis). Surgical excision of the lesion is the elective treatment.
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PMID:Intraosseous xanthoma without lipid disorders. Case-report and literature review. 1077 72

Pulmonary Langerhans' cell histiocytosis and eosinophilic granuloma are the terms used to describe a Langerhans' cell granulomatous interstitial lung disease of unknown aetiology, occurring predominantly in smokers and involving primarily lungs, bones, skin and lymph nodes. In this report a patient with fever, fatigue, dyspnoea, nocturnal perspiration and thoracic pain is described. The high-resolution computed tomography of the chest and histological examination of lung biopsies suggested the diagnosis of pulmonary Langerhans' cell histiocytosis. The disease was limited to the lung, since further investigations did not show any other localization. The patient had a good clinical outcome with avoidance of smoking and steroid therapy. The computed tomography scan follow-up showed a partial resolution of pulmonary lesions.
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PMID:Favourable outcome of a case of pulmonary Langerhans' cell histiocytosis. 1078 15

Eosinophilic granuloma of the jaws is a rather benign and localized form of Langerhans' cell histiocytosis. Treatment is usually required in larger lesions that cause local pain and swelling and pose the risk of spontaneous fractures. There are several accepted forms of treatment, which include surgery, radiation therapy, systemic and local therapy with corticoids, and systemic chemotherapy. No studies exist that compare the effectiveness of these treatment modalities. We report a novel therapeutic regimen that uses repeated intraosseous injections of triamcinolone-1 16 alpha 21-diacetat, a synthetic corticoid, which led to a rapid, complete, and durable treatment. The patient had a multilocal eosinophilic granuloma of the mandible in which radiation therapy, systemic corticoid therapy, and systemic chemotherapy had failed.
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PMID:Multifocal eosinophilic granuloma of the jaw: long-term follow-up of a novel intraosseous corticoid treatment for recalcitrant lesions. 1098 53

We report a 24-years-old woman who presented with thoracic pain after coughing. Physical exam revealed no abnormalities except pain after pressing under blade-bone area. A rib radiography and CT of the thorax showed a lonely osteolytic lesion inside eleventh left costal arch without affecting others tissues. There were no more osteolytic lesions at other levels and the histopathological study of a resection-biopsy of the lesion was diagnosed as Langerhans' cell granulomatosis. This is an uncommon disease which diagnosis can only be made through histopathological study of suspected lesions.
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PMID:[Unifocal Langerhans cell granulomatosis in a young woman]. 1110 May 38

Langerhans' cell histiocytosis is a disease of the dendritic histiocytes with a wide variety of clinical manifestations. This report describes a boy with Langerhans' cell histiocytosis who presented with primarily neurologic and endocrinologic findings, without pain. The diagnosis of Langerhans' cell histiocytosis was not made until 10 years after symptom onset. The pathology database at Mayo Clinic was searched for cases of Langerhans' cell histiocytosis between 1985 and 1999 under 19 years of age (65 children), and information regarding clinical presentation was abstracted. Database review found a range of 1 day to 156 weeks (mean 13.8 weeks) from symptom onset to diagnosis. No other patients with primarily neurologic symptoms were found. The diagnosis of Langerhans' cell histiocytosis was made significantly sooner after onset if pain was present (chi-square = 19.1, P < .001, two-tailed, phi coefficient 0.54). Our findings indicate that neurologic manifestations of Langerhans' cell histiocytosis are rare, and the combination of diabetes insipidus, ataxia, skin rash, or osseous pain should alert the clinician to the possibility of Langerhans' cell histiocytosis and avoid delayed diagnosis.
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PMID:Delayed diagnosis of pediatric Langerhans' cell histiocytosis: case report and retrospective review of pediatric cases seen at Mayo Clinic. 1145 55

A 63-year old man had a history of diabetes insipidus, arthralgias and myalgias, weight loss, relapsing fever and malaise. Increased uptake of Tc-99m was found predominantly in distal antebrachia, in distal femurs and in both trochanters and tibias on the bone scintigraphy. The chest radiograph showed reticulonodular pattern and the high resolution computed tomography (HRCT) scans revealed diffuse infiltrative lung disease with small multiple nodules and widening of interlobular septs. Videothoracoscopic lung biopsy and biopsy of tibial lesion were perfomed. The histopathologic examination proved non-Langerhans cell histiocytosis-Erdheim-Chester disease. Treatment with prednisone reduced the pain and fever and improved the vital capacity of the lungs while the changes in the lungs and bones remained unchanged.
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PMID:Erdheim-Chester disease. A case report. 1149 97

A 48-year-old woman presented with a history of premature menopause, polyuria, polydipsia, fever, and diffuse bony tenderness. Her evaluation revealed central diabetes insipidus, hypothalamic amenorrhea, an elevated free calcium on multiple occasions with an elevated 1,25 dihydroxyvitamin D level, and osteoporosis by densitometry. Skeletal series revealed multiple lytic lesions involving the long bones. The diagnosis of Langerhans' cell granulomatosis was made. She was treated with hormone replacement therapy, radiotherapy, and vinblastine, with a dramatic improvement in her pain and a near normalization of her free calcium. Whereas hypercalcemia has been described in several granulomatous disorders and is secondary to unregulated extrarenal production of 1,25 dihydroxyvitamin D, it is, however, extremely rare in Langerhans' cell granulomatosis. This is the first case report of Langerhans' cell granulomatosis with hypercalcemia and documented elevated increased 1,25 dihydroxyvitamin D level that responded to the treatment of her primary disease.
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PMID:Hypercalcemia in Langerhans' cell granulomatosis with elevated 1,25 dihydroxyvitamin D (calcitriol) level. 1179 6

The histiocytic syndromes consist of a group of disorders that share in common the proliferation of cells of the monocytic/macrophage lineage. It has been conventional to divide the histiocytoses into two separate groups: Langerhans cell histiocytosis (LCH) and non-LCH. We present a 2-year-old Hispanic boy who was referred to the dermatology clinic for evaluation of an asymptomatic cutaneous eruption of the head and upper trunk. In addition, he had a 3-week history of pain in his right leg and difficulty in walking. The patient's physical examination was normal, excluding the skin findings. On plain radiography, multiple lytic lesions in the skull, lumbar spine, and right tibia were seen. Histopathologic examination of a skin biopsy specimen revealed a predominantly histiocytic infiltrate in the dermis which was negative for S-100 and CD1a stains. A tibial biopsy specimen showed a monomorphous infiltrate of histiocytes that were S-100 and CD1a positive. This patient's concomitant findings of both LCH and non-LCH histiocytoses further support a potential overlap within the histiocytic syndromes, as has been suggested by others.
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PMID:A child with both Langerhans and non-Langerhans cell histiocytosis. 1238 99


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