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)

Histiocytosis X is very rare in late adulthood, usually taking a benign course with a good prognosis. A 56-year-old patient developed acute disseminated histiocytosis X with multiorgan involvement. Despite extensive diagnostic work-up the diagnosis could only be established on the grounds of a skin biopsy. Histiocytosis X cells are S 100 protein-positive and express the membrane antigens CD 1, CD 4 and HLA-DR. Electron microscopy reveals Birbeck granules. On static cytophotometry, histiocytosis X cells exhibited normal DNS content without aneuploidy. One year after the onset of disease the patient's condition deteriorated rapidly despite aggressive polychemotherapy (CHOP), and he died of multiorgan failure due to histiocytosis X.
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PMID:[Acute disseminated histiocytosis X (Langerhans cell histiocytosis) with fatal course in an adult]. 191 71