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Lymphoproliferative Syndrome 1

Disease ID: disease_node_20435

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DbxrefICD10CM:D47.9, MIM:613011
SubclassofDOID_0050737, DOID_0060704
Data SourceDOID
SynonymsLPFS1
Doid Labellymphoproliferative syndrome 1
Doid DescriptionA lymphoproliferative syndrome characterized by autosomal recessive inheritance, early childhood onset of Epstein-Barr virus-associated immune dysregulation typically manifesting as lymphoma, lymphomatoid granulomatosis, hemophagocytic lymphohistiocytosis, Hodgkin disease, and-or hypogammaglobulinemia and that has_material_basis_in homozygous mutation in the ITK gene on chromosome 5q32.
Existence Starts DuringHP_0011463
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_20435
Doid IdDOID_0060707
LabelLymphoproliferative Syndrome 1