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X-linked lymphoproliferative syndrome 1
Summary
- Synonym
-
- XLP1
- Definition
- A lymphoproliferative syndrome characterized by severe immune dysregulation after viral infection that may manifest as severe or fatal mononucleosis, acquired hypogammaglobulinema, hemophagocytic lymphohistiocytosis, and/or malignant lymphoma and that has_material_basis_in X-linked mutation in the SH2D1A gene on chromosome Xq25.
- Super Class
- X-linked recessive disease lymphoproliferative syndrome
