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)

Hemophagocytic lymphohistiocytosis, terminology that designates a syndrome that may be familial or sporadic, with or without an associated viral infection, is presented as the prototype of a hemophagocytic syndrome, a condition in which there is uncontrolled activation of the cellular immune system. Diagnostic criteria include idiopathic fever, splenomegaly, cytopenias, hypertriglyceridemia, hypofibrinogenemia, and the presence of hemophagocytosis. The surgical and autopsy pathology features infiltrates composed of lymphocytes and ordinary, but activated, histiocytes and hemophagocytosis. The chronic hepatitis-like hepatic lesion is noted to be characteristic, if not unique, in this age group and setting. Current concepts of pathophysiology focus on the role of cytokines, particularly interleukin (IL)-1, IL-2, soluble IL-2 receptor, plasminogen activator, and prostaglandins. The clinicopathologic features of the syndrome can be accounted for by the uncontrolled and unopposed production and release of these mediators. Nosology places hemophagocytic lymphohistiocytosis in the position of the most important of the "benign" histiocytosis syndromes that involve ordinary histiocytes of the mononuclear phagocytic system in contrast to Langerhans cell histiocytosis (histiocytosis X) in which pathological dendritic histiocytes are operative. Features that distinguish hemophagocytic lymphohistiocytosis from other disorders, such as malignant histiocytosis, X-linked lymphoproliferative disorder, congenital immunodeficiency states, the accelerated phase of Chediak-Higashi syndrome, and cytophagic histiocytic panniculitis, which may be associated with a hemophagocytic syndrome, are presented.
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PMID:Hemophagocytic lymphohistiocytosis: a hemophagocytic syndrome. 156 89

The authors describe a case of a 42-year-old woman with systemic xanthogranulomatosis who primarily manifested bilateral intraorbital tumors, generally effecting many regions. These included the intracranial dura mater, the spinal cord, the retroperitoneum, the pericardium, and the mediastinum. This disorder is basically similar to systemic Weber-Christian disease except for the absence of subcutaneous panniculitis. Immunohistochemical study reveals that this clinical entity is different from histiocytosis X because the origin of the foamy cells is not the T-zone histiocyte but the macrophage. The treatment of this disease is also discussed.
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PMID:[A case of systemic xanthogranulomatosis with an intracranial lesion: immunohistochemical study]. 202 77

The authors describe a 42-year-old woman with systemic xanthogranulomatosis and bilateral intraorbital tumors, who subsequently developed multiple lesions of the intracranial dura mater, spinal cord, retroperitoneum, pericardium, and mediastinum. Systemic xanthogranulomatosis is histologically similar to systemic Weber-Christian disease, except for the absence of subcutaneous panniculitis. Immunohistochemical studies suggest that this clinical entity can be differentiated from histiocytosis X, because foamy cells in systemic xanthogranulomatosis demonstrate macrophages but not T-zone histiocyte markers. Differentiation of this disease from other intracranial xanthogranulomas and treatment are discussed.
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PMID:An intracranial mass lesion in systemic xanthogranulomatosis: case report. 217 1

Subcutaneous fat necrosis of the newborn (SCFNN) is a rare form of panniculitis classically affecting healthy full-term infants. There are a number of predisposing factors including perinatal asphyxia. The condition generally has a benign course with spontaneous resolution, but monitoring for metabolic complications, in particular the potentially life-threatening complication of hypercalcaemia, is critical. The authors report 2 cases of preterm infants with perinatal asphyxia with atypical presentations of SCFNN: the first with bony involvement resembling Langerhans cell histiocytosis and with follicular pseudocarcinomatous hyperplasia on histology; and the second presenting with a huge haematoma requiring surgical debridement. Both cases were initially erroneously diagnosed as pyogenic infections.
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PMID:Report of 2 Novel Presentations of Subcutaneous Fat Necrosis of the Newborn. 3170 Aug 55