Epstein-Barr virus infections in the X-linked recessive lymphoproliferative syndrome
| Autor: | David A. Thorley-Lawson, JamesP.S. Yang, Jag Bhawan, William Boto, Rorert Maier, LindseyM. Hutt, I.O. Szymanski, DavidT. Purtilo, Lucian K. Denicola |
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| Rok vydání: | 1978 |
| Předmět: |
Male
Herpesvirus 4 Human X Chromosome Adolescent Genetic Linkage Lymphoproliferative disorders Genes Recessive Biology Environment Measles Immune system Agammaglobulinemia hemic and lymphatic diseases medicine Humans Infectious Mononucleosis Prospective Studies Epstein–Barr virus infection X-linked recessive inheritance X chromosome Pneumonitis Subclinical infection General Medicine medicine.disease Virology Burkitt Lymphoma Lymphoproliferative Disorders Phenotype Immunology Female Lymphoma Large B-Cell Diffuse |
| Zdroj: | Lancet (London, England). 1(8068) |
| ISSN: | 0140-6736 |
| Popis: | Prospective studies demonstrated variable phenotypic expression of the X-linked recessive lymphoproliferative syndrome (X.L.R.L.S.) in three brothers: (1) hypogammaglobulinaemia and subclinical Epstein-Barr-virus (E.B.V.) infection with antibody response to E.B.V.; (2) E.B.V. infection with defective immune response to E.B.V., fatal infectious mononucleosis (I.M.), and immunoblastic lymphoma; and (3) histiocytic lymphoma. Hypogammaglobulinaemia and measles pneumonitis had preceded infection with E.B.V. The diverse phenotypic expressions probably resulted from the varied immune response to E.B.V. Recombination of X chromosomes was documented by Xg-blood-group studies in a survivor. E.B.V. can induce fatal I.M. and malignant lymphoma in X.L.R.L.S., but an immune response to E.B.V. can be protective. |
| Databáze: | OpenAIRE |
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