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 9 month-old boy developed secondary open-angle glaucoma associated with fever and multiple lytic bone lesions 2 1/2 months after cataract aspiration. An open bone biopsy provided a diagnosis of histiocytosis X. Hisiocytes similar to those in the bone were identified in a specimen of aqueous humor from the affected eye. No other cells were found. The abnormal histiocytes may have caused the glaucoma by infiltrating the trabecular angle structures and mechanically obstructing aqueous outflow from the eye.
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PMID:Secondary open-angle glaucoma in histiocytosis X. 30 48

Cutaneous histiocytosis may take two principal forms. It is either a benign proliferative process or a relentless, progressive process with a poor prognosis. In histiocytic medullary reticulosis, histiocytes demonstrate nuclear atypia and the outcome is uniformly fatal. Benign cephalic histiocytosis X causes lesions similar to those of histiocytosis X, but Langerhans' cells are absent. In congenital self-healing histiocytosis X, the Letterer-Siwe-like cutaneous infiltrate contains Langerhans' cells, but the lesions heal spontaneously without treatment. The nodular cutaneous lesions of juvenile xanthogranuloma appear in infancy and resolve without treatment; however, the higher percentage (10%) of associated ocular lesions may lead to glaucoma and blindness. In histiocytosis X, the cutaneous lesions show a marked proliferation of Langerhans' cells, with prognosis dependent on the patient's age and the extent of organ dysfunction. Patients who survive the acute form of the disease may develop diabetes insipidus, growth retardation, pulmonary fibrosis, and biliary cirrhosis. A subtle immunologic defect has been identified in patients with histiocytosis X, yet the pathogenesis of the disease is still speculative. Familial disease occurring in early infancy should be differentiated from complete or partial immunodeficiency syndromes. Guidelines for evaluating patients with cutaneous histiocytosis are reviewed.
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PMID:Cutaneous histiocytosis syndromes. 299 40

A 6-year-old boy with known multisystem Langerhans cell histiocytosis developed photophobia, conjunctival injection, iris neovascularization, and an iridociliochoroidal mass. Fine-needle aspiration biopsy revealed mononucleated and multinucleate histiocytes that demonstrated positive immunostaining for CD68 and S100 consistent with Langerhans cell histiocytosis. Management with intracameral bevacizumab (1.25 mg/0.05 mL) resolved the iris neovascularization, and plaque radiotherapy (brachytherapy) resolved the mass rapidly and completely, preserving the patient's visual acuity and preventing glaucoma during the 10-month follow-up.
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PMID:Langerhans cell histiocytosis of the uvea with neovascular glaucoma: diagnosis by fine-needle aspiration biopsy and management with intraocular bevacizumab and brachytherapy. 2116 78

Juvenile xanthogranuloma (JXG) is a benign histiocytic skin disorder mainly encountered during infancy and childhood. Although with multiple potential localizations, less than 1% of the cases exhibit ocular manifestations. Some of these might lead to serious complications, specifically, secondary glaucoma that can result in severe and blinding eye disease. The aim of the present case report was to demonstrate typical clinical features, emphasize the difficulties attributed when managing these patients and literature review. We present the case of 4 months old female baby with spontaneous hyphema and secondary unilateral glaucoma due to ocular JXG. The natural history and treatment of the condition were extremely difficult to handle due to multiple opinions in histopathology related to other severe conditions that resembled with the lesions detected in this case: myelomonocytic leukemia and Langerhans cell histiocytosis. Although a minority of patients with JXG have ocular involvement, recognition of this condition is important because a treatment delay can lead to serious complications, such as glaucoma and spontaneous hyphema, as in our case. A thorough differential diagnosis represents the key to a proper management plan in these patients, both on short and long term. "Triple disease" defined as JXG plus neurofibromatosis type 1 (NF-1) and juvenile chronic myelogenous leukemia (JCML) has been reported, but it was not confirmed in our patient.
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PMID:Iris juvenile xanthogranuloma in an infant - spontaneous hyphema and secondary glaucoma. 2945 Apr 3