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)

The Histiocytoses are defined as non-malignant disorders due to abnormal accumulation and behavior of cells of the mononuclear phagocytic system. The best known histiocytoses, Langerhans cell histiocytosis (LCH), and hemophagocytic lymphohistiocytosis (HLH), each with an estimated incidence of 1/50,000 to 1/150,000, are sufficiently "common," complex and costly, to constitute an important problem in medical practice. At the same time, LCH, HLH and an array of other and more rare histiocytoses are sufficiently uncommon that most physicians lack the experience to diagnose, let alone care for patients with these conditions. The pathophysiology of most of the histiocytoses is unknown and, in the case of the widely-disseminated and potentially fatal forms, treatments to date have been variably effective and sometimes highly toxic. MAS has been reported to occur in association with almost any rheumatic disease, it is by far most common in the systemic form of Juvenile Idiopthic Arthritis (SoJIA). It is now recognized that MAS bears a close resemblance to Hemophagocytic Lymphohistiocytosis or HLH, and MAS is recognized as the major fatal complication of soJIA.
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PMID:Histiocytic disorders: recent insights into pathophysiology and practical guidelines. 1993 59

Fibrous dysplasia (FD) is categorized as either monostotic or polyostotic and may occur as a component of McCune-Albright syndrome (MAS). Imaging findings can mimic neoplastic diseases. We present a case of MAS initially suspected to have neoplastic disease. A 9-year-old girl was admitted to pediatric emergency with ataxia. Upon hospitalization, an extradural mass was seen on cranial magnetic resonance imaging (MRI) and the bone survey showed lytic lesions in the long bones. The patient was referred to the pediatric oncology department with a presumptive diagnosis of Langerhans cell histiocytosis or metastatic tumor. Further investigations demonstrated that the patient had MAS and coexisting postinfectious cerebellitis. The findings in this patient demonstrate that the radiographic findings and the clinical presentation of FD and MAS may be similar to those of malignant diseases.
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PMID:McCune-Albright syndrome mimicking malignancy: an endocrine disease from oncologist's perspective. 2298 16