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X-Linked Lymphoproliferative Syndrome 1

Disease ID: disease_node_20436

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DbxrefGARD:7906, ICD10CM:D82.3, MIM:308240
SubclassofDOID_0080012, DOID_0060704
Data SourceDOID
SynonymsXLP1
Doid LabelX-linked lymphoproliferative syndrome 1
Doid DescriptionA lymphoproliferative syndrome characterized by severe immune dysregulation after viral infection that may manifest as severe or fatal mononucleosis, acquired hypogammaglobulinema, hemophagocytic lymphohistiocytosis, and/or maligt lymphoma and that has_material_basis_in X-linked mutation in the SH2D1A gene on chromosome Xq25.
Has Material Basis InGENO_0000149
Disease Node Iddisease_node_20436
Doid IdDOID_0060705
LabelX-Linked Lymphoproliferative Syndrome 1