Langerhans'-Cell Histiocytosis (Histiocytosis X) -- A Clonal Proliferative Disease
| Autor: | B E Favara, L Busque, Kenneth L. McClain, M H Duncan, Cheryl L. Willman, B B Griffith, D G Gilliland |
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| Rok vydání: | 1994 |
| Předmět: |
Pathology
medicine.medical_specialty biology business.industry General Medicine Adult Langerhans Cell Histiocytosis medicine.disease Lesion Histiocytosis Langerhans cell histiocytosis Immunology medicine biology.protein Langerhans cell sarcoma Disseminated disease Antibody medicine.symptom business Histiocyte |
| Zdroj: | New England Journal of Medicine. 331:154-160 |
| ISSN: | 1533-4406 0028-4793 |
| Popis: | Background The lesions of Langerhans'-cell histiocytosis (histiocytosis X), a proliferative histiocytic disorder of unknown cause, contain histiocytes similar in phenotype to dendritic Langerhans' cells. The disease ranges in severity from a fatal leukemia-like disorder to an isolated lytic lesion of bone. Intermediate forms of the disease are usually characterized by multiorgan involvement, diabetes insipidus, and a chronic course. Methods To determine whether Langerhans' histiocytosis is a polyclonal reactive disease or a clonal disorder, we used X-linked polymorphic DNA probes (HUMARA, PGK, M27β[DXS255], and HPRT) to assess clonality in lesional tissues and control leukocytes from 10 female patients with various forms of the disease. Lymphoid clonality was also assessed by analysis of rearrangements at immunoglobulin and T-cell-receptor gene loci. Results The HUMARA assay detected clonal cells in the lesions of 9 of the 10 patients: 3 patients had acute disseminated disease, 3 had unifocal disease, and... |
| Databáze: | OpenAIRE |
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