Lymphoproliferative Disease Associated with Primary Immune Disorder

Preferred Label : Lymphoproliferative Disease Associated with Primary Immune Disorder;

NCIt synonyms : Lymphoproliferative Disease Associated with PID;

NCIt definition : A lymphoid proliferation that arises in the setting of immune deficiency due to a primary immunodeficiency or immunoregulatory disorder. The primary immune disorders most frequently associated with lymphoproliferative disorders are ataxia-telangiectasia, Wiskott-Aldrich syndrome, common variable immunodeficiency, severe combined immunodeficiency, X-linked lymphoproliferative disease, Nijmegen breakage syndrome, hyper-IgM syndrome, and autoimmune lymphoproliferative syndrome. (WHO 2017);

NCI Metathesaurus CUI : CL552565;

Details

Origin ID

C150673;

UMLS CUI

C4725913;

Disease excludes normal cell origin
- Myeloid Cell [NCIt concept]
Has associated anatomic sites
- Hematopoietic and Lymphatic System [NCIt concept]
Semantic type(s)
- Disease or Syndrome [UMLS semantic type]
disease_has_abnormal_cell
- Abnormal Lymphocyte [NCIt concept]
disease_has_associated_disease
- Congenital Combined Immunodeficiency [NCIt concept]
disease_has_finding
- Immunodeficiency [NCIt concept]
disease_has_normal_cell_origin
- Hematopoietic and Lymphoid Cell [NCIt concept]
- Lymphocyte [NCIt concept]
disease_has_normal_tissue_origin
- Hematopoietic and Lymphoid Tissue [NCIt concept]
- Lymphoid Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Hematopoietic and Lymphatic System [NCIt concept]
- Lymphatic System [NCIt concept]
may_be_associated_disease_of_disease
- Adenosine Deaminase Deficiency [NCIt concept]
- Ataxia Telangiectasia Syndrome [NCIt concept]
- Autoimmune Lymphoproliferative Syndrome [NCIt concept]
- Autoimmune Lymphoproliferative Syndrome with FAS Mutation [NCIt concept]
- Autoimmune Lymphoproliferative Syndrome with Germline CASP10 Mutation [NCIt concept]
- Autoimmune Lymphoproliferative Syndrome with Germline CASP8 Mutation [NCIt concept]
- Autoimmune Lymphoproliferative Syndrome with Germline FADD Mutation [NCIt concept]
- Autoimmune Lymphoproliferative Syndrome with Germline FAS Mutation [NCIt concept]
- Autoimmune Lymphoproliferative Syndrome with Germline FASLG Mutation [NCIt concept]
- Autoimmune Lymphoproliferative Syndrome with Other Specified FAS Pathway Germline Mutation [NCIt concept]
- Autoimmune Lymphoproliferative Syndrome with Somatic FAS Mutation [NCIt concept]
- Autoimmune Lymphoproliferative Syndrome-Unknown Underlying Mutation [NCIt concept]
- Bare Lymphocyte Syndrome [NCIt concept]
- Bare Lymphocyte Syndrome Type 1 [NCIt concept]
- Bare Lymphocyte Syndrome Type 2 [NCIt concept]
- Common Variable Immunodeficiency [NCIt concept]
- IL-7R-Alpha-deficient Severe Combined Immunodeficiency [NCIt concept]
- Immunodeficiency 26 with or without Neurologic Abnormalities [NCIt concept]
- Immunodeficiency, Common Variable, 11 [NCIt concept]
- Immunodeficiency, Common Variable, 8, with Autoimmunity [NCIt concept]
- JAK3-deficient Severe Combined Immunodeficiency [NCIt concept]
- Leaky Severe Combined Immunodeficiency [NCIt concept]
- MHC Class II Deficiency [NCIt concept]
- Nijmegen Breakage Syndrome [NCIt concept]
- Omenn Syndrome [NCIt concept]
- Reticular Dysgenesis [NCIt concept]
- Severe Combined Immunodeficiency [NCIt concept]
- Severe Combined Immunodeficiency due to CD45 Deficiency [NCIt concept]
- Severe Combined Immunodeficiency due to NHEJ1 Deficiency [NCIt concept]
- Severe Combined Immunodeficiency due to RAG Deficiency [NCIt concept]
- Severe Combined Immunodeficiency due to RAG1 Deficiency [NCIt concept]
- Severe Combined Immunodeficiency due to RAG2 Deficiency [NCIt concept]
- Severe Combined Immunodeficiency with Absence of T and B Cells [NCIt concept]
- Severe Combined Immunodeficiency with Absence of T, B, and NK Cells [NCIt concept]
- Severe Combined Immunodeficiency with Absence of T, Normal B Cells [NCIt concept]
- Severe Combined Immunodeficiency, Athabascan Type [NCIt concept]
- Severe Combined Immunodeficiency, Autosomal Recessive, T-Cell Negative, B Cell-Positive, NK Cell-Positive [NCIt concept]
- Wiskott-Aldrich Syndrome [NCIt concept]
- X-Linked Lymphoproliferative Syndrome [NCIt concept]
- X-Linked Severe Combined Immunodeficiency [NCIt concept]
- X-linked Lymphoproliferative Syndrome 1 [NCIt concept]
- X-linked Lymphoproliferative Syndrome 2 [NCIt concept]

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