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x-linked lymphoproliferative syndrome

Medical Definition

An x-linked immunodeficiency syndrome that exclusively affects males, although females can be carriers. it is caused by mutation(s) in sh2d1a and/or xiap genes and is characterized by life-threatening episodes of infectious mononucleosis, hypogammaglobulinemia, and subsequent development of lymphomas (usually b-cell lymphomas) and other lymphoproliferative disorders.
Related Codes (1)
Code
Description
Billable
Details
D82.3Immunodeficiency following hereditary defective response to Epstein-Barr virus

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