X-linked lymphoproliferative syndrome

X-linked lymphoproliferative syndrome
a hereditary disorder of the immune system caused by a defective sex-linked gene carried on an X chromosome. There is uncontrolled proliferation of B-lymphocyte in response to infection by the Epstein-Barr virus, which can lead to fulminating hepatitis or lymphoma. This condition is due to a defect in a gene, SAP, which encodes a signalling molecule found in the cytoplasm of cells.

Medical dictionary. 2011.

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  • X-linked lymphoproliferative syndrome — XLP syndrome; = Duncan s disease a hereditary disorder of the immune system caused by a defective sex linked gene carried on an X chromosome. There is uncontrolled proliferation of B lymphocytes in response to infection by the Epstein Barr virus …   The new mediacal dictionary

  • X-linked lymphoproliferative syndrome — an X linked immunodeficiency disorder caused by mutation in either the SHD2D1A or XIAP gene (both at locus Xq25), which are involved in T cell development and apoptosis; the disorder is characterized by defective cellular or humoral immune… …   Medical dictionary

  • X-linked lymphoproliferative disease — Classification and external resources ICD 10 D82.3 OMIM 308240 300635 …   Wikipedia

  • X-linked lymphoproliferative disease — see under syndrome …   Medical dictionary

  • Lymphoproliferative disorders — Infobox Disease Name = PAGENAME Caption = DiseasesDB = ICD10 = ICD9 = ICD9|238.79 ICDO = OMIM = MedlinePlus = eMedicineSubj = ped eMedicineTopic = 1345 MeshID = D008232 Lymphoproliferative disorders (LPDs) refer to several conditions in which… …   Wikipedia

  • syndrome — The aggregate of symptoms and signs associated with any morbid process, and constituting together the picture of the disease. SEE ALSO: disease. [G. s., a running together, tumultuous concourse; (in med.) a concurrence of symptoms, fr. syn,… …   Medical dictionary

  • Duncan syndrome — Dun·can syndrome (dungґkən) [Duncan, the original kindred in which the disease was described] X linked lymphoproliferative syndrome; see under syndrome …   Medical dictionary

  • Purtilo syndrome — Pur·ti·lo syndrome (pər teґlo) [David T. Purtilo, American physician, 20th century] X linked lymphoproliferative syndrome; see under syndrome …   Medical dictionary

  • XLP syndrome — see X linked lymphoproliferative syndrome …   Medical dictionary

  • XLP syndrome — see X linked lymphoproliferative syndrome …   The new mediacal dictionary

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