X-linked lymphoproliferative syndrome

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(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, 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.

Subjects: Medicine and Health.

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