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 1 ID (Ontology) DOID:0060705 (Human Disease)
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.
Also Known As "XLP1"
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X-linked monogenic disease
 |__X-linked recessive disease____
primary immunodeficiency disease  |
 |__lymphoproliferative syndrome__|
                                  X-linked lymphoproliferative syndrome 1
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Is a lymphoproliferative syndrome
X-linked recessive disease
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Synonyms
  • "XLP1" EXACT OMO:0003012
Secondary IDs
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GARD:7906
ICD10CM:D82.3
MIM:308240