FB2025_05 , released December 11, 2025
FB2025_05 , released December 11, 2025
CV Term Report
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General Information
Term X-linked lymphoproliferative syndrome 2 ID (Ontology) DOID:0060706 (Human Disease)
Definition A lymphoproliferative syndrome characterized by X-linked inheritance, immune dysregulation after viral infect that may include lymphohystiocytosis, hypogammaglobulinemia and/or splenomegaly and that has_material_basis_in mutation in the XIAP gene on chromosome Xq25.
Also Known As "XIAP deficiency" ; "XLP2"
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 Genes
 X-linked lymphoproliferative syndrome 2       1
 for disease ribbon | X-linked lymphoproliferative syndrome 2       1
 model of | X-linked lymphoproliferative syndrome 2       1
Spanning Tree (Parents/Children)
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X-linked monogenic disease
 |__X-linked recessive disease____
primary immunodeficiency disease  |
 |__lymphoproliferative syndrome__|
                                  X-linked lymphoproliferative syndrome 2  1 rec.
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Is a lymphoproliferative syndrome
X-linked recessive disease
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Synonyms
  • "XIAP deficiency" EXACT
    "XLP2" EXACT OMO:0003012
Secondary IDs
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GARD:10916
ICD10CM:D82.3
MIM:300635