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)

Eosinophilic granuloma of bone is the localised and most benign form of Langerhans-cell histiocytosis, previously known as histiocytosis X and is characterised by lytic lesions of one or more bones. It is a disease of children and adolescents, and very rarely affects adults. We report a 34-year-old patient with a solitary eosinophilic granuloma involving the fifth lumbar vertebra which produced sciatica. Operative treatment performed by a single extended posterior approach consisted of total resection of the tumour with hemicorporectomy, L4-5 and L5-S1 discectomy, interbody fusion with an iliac bone graft, and interpedicular fixation. Postoperatively, the symptoms resolved and the patient continues to do well at 2 1/2 years follow-up.
Paraplegia 1995 Aug
PMID:Solitary eosinophilic granuloma of the lumbar spine in an adult. Case report. 747 47

Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis. It is characterized by osteosclerosis of the metaphyseal regions of long bones and several extraskeletal manifestations. Clinically, it ranges from an asymptomatic focal process to systemic disease with life-threatening visceral involvement. Until now, only two cases of Erdheim-Chester disease with paraparesis have been reported. Herein we report the first case of Erdheim-Chester disease with the clinical manifestation of paraplegia. Our patient also had diabetes insipidus, pleural and pericardial effusion, retro-orbital and cavernous sinus masses, fibrotic changes in the retroperitoneal, perirenal, and periaortic areas, and epidural space-occupying lesions. We want to emphasize that ECD may be a very rare cause of paraplegia.
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PMID:Erdheim-Chester disease: a rare cause of paraplegia. 1255 12

Langerhans cell histiocytosis (LCH) is a clonal proliferation of Langerhans cells occurring as an isolated lesion or as part of a systemic proliferation. It is commoner in children younger than 10 years of age with sparing of the posterior elements in more than 95% of cases. We describe a case of LCH in an adult female presenting with paraplegia. MRI revealed a well-defined extradural contrast enhancing mass at D2-D4 vertebral level involving the posterior elements of spine. D2-5 laminectomy with excision of lesion was performed which lead to marked improvement of patients neurological status. Histopathology was suggestive of eosinophilic granuloma. We describe the case, discuss its uniqueness and review the literature on this rare tumor presentation.
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PMID:Langerhans' cell histiocytosis involving posterior elements of the dorsal spine: An unusual cause of extradural spinal mass in an adult. 2312 97