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Term:
Parent Terms Term With Siblings Child Terms
agammaglobulinemia +   
An immunologic deficiency state characterized by an extremely low level of generally all classes of gamma-globulin in the blood.
alpha chain disease 
Anhaptoglobinemia  
Anhidrotic Ectodermal Dysplasia with Immunodeficiency, Osteopetrosis, and Lymphedema  
Anhidrotic Ectodermal Dysplasia with T-Cell Immunodeficiency, Autosomal Dominant  
Antibody Deficiency due to Defect in CD19 
antithrombin III deficiency  
ataxia telangiectasia +   
autoimmune lymphoproliferative syndrome +   
Autoinflammation, Antibody Deficiency, and Immune Dysregulation, PLCG2-Associated  
B cell deficiency +   
B-Cell Immunodeficiency, Distal Limb Anomalies, and Urogenital Malformations 
C1q Deficiency  
C9 Deficiency with Dermatomyositis 
CARD11 Immunodeficiency  
Cartilage Hair Hypoplasia Like Syndrome 
cartilage-hair hypoplasia  
Castleman disease +   
Cd4+ Lymphocyte Deficiency  
CD8 Deficiency, Familial  
COMBINED IMMUNODEFICIENCY AND MEGALOBLASTIC ANEMIA WITH OR WITHOUT HYPERHOMOCYSTEINEMIA  
Combined Immunodeficiency with Autoimmunity and Spondylometaphyseal Dysplasia  
Combined Inflammatory and Immunologic Defect 
combined T cell and B cell immunodeficiency +   
common variable immunodeficiency +   
complement component 9 deficiency  
complement deficiency +   
Congenital Erythroderma with Palmoplantar Keratoderma, Hypotrichosis, and Hyper-IgE  
congenital hypogammaglobulinemia 
Corticosteroid-Binding Globulin, Elevated 
Davenport Donlan Syndrome 
Deltaretrovirus Infections +   
dysgammaglobulinemia +   
Endotoxin Hyporesponsiveness  
EVANS SYNDROME, IMMUNODEFICIENCY, AND PREMATURE IMMUNOSENESCENCE ASSOCIATED WITH TRIPEPTIDYL-PEPTIDASE II DEFICIENCY  
Facial Dysmorphism, Immunodeficiency, Livedo, and Short Stature  
familial cold autoinflammatory syndrome +   
Familial Enteropathy with Villous Edema and Immunoglobulin G2 Deficiency 
Fanconi-like syndrome  
Glucose-Stimulated Secretory Diarrhea, with Common Variable Immunodeficiency 
Granuloma +   
Griscelli syndrome +   
hairy cell leukemia +   
heavy chain disease +  
Hepatic Venoocclusive Disease with Immunodeficiency  
human immunodeficiency virus infectious disease +   
Hypergammaglobulinemia +   
Hypergastrinemia  
hyperimmunoglobulin syndrome +   
Hypoglobulinemia and Absent B Cells 
Hypoproteinemia +   
IL21R Immunodeficiency  
Immune Deficiency Disease  
Immune Deficiency, Familial Variable 
Immunoblastic Lymphadenopathy  
IMMUNODEFICIENCY 11B WITH ATOPIC DERMATITIS  
Immunodeficiency 12  
Immunodeficiency 14  
IMMUNODEFICIENCY 15A  
Immunodeficiency 15B  
Immunodeficiency 16  
Immunodeficiency 19  
Immunodeficiency 20  
Immunodeficiency 21  
Immunodeficiency 22  
Immunodeficiency 23  
IMMUNODEFICIENCY 24  
IMMUNODEFICIENCY 26 WITH OR WITHOUT NEUROLOGIC ABNORMALITIES  
Immunodeficiency 27B  
IMMUNODEFICIENCY 28  
IMMUNODEFICIENCY 29  
Immunodeficiency 30  
Immunodeficiency 31A  
IMMUNODEFICIENCY 31B  
Immunodeficiency 31C  
Immunodeficiency 32A  
Immunodeficiency 32B  
Immunodeficiency 36  
Immunodeficiency 37  
Immunodeficiency 38, with Basal Ganglia Calcification  
Immunodeficiency 39  
Immunodeficiency 40  
Immunodeficiency 42  
Immunodeficiency 44  
Immunodeficiency 45  
Immunodeficiency 46  
Immunodeficiency 47  
Immunodeficiency 48  
Immunodeficiency 49  
Immunodeficiency 50  
Immunodeficiency 51  
Immunodeficiency 52  
Immunodeficiency 53  
Immunodeficiency 55  
Immunodeficiency 57  
Immunodeficiency 58  
Immunodeficiency 60  
Immunodeficiency 62  
Immunodeficiency 63 with Lymphoproliferation and Autoimmunity  
Immunodeficiency 64  
Immunodeficiency 65 
Immunodeficiency due to Defect in CD3-Epsilon  
Immunodeficiency due to Defect in CD3-Gamma  
Immunodeficiency due to Defect in CD3-Zeta  
Immunodeficiency due to Defect in MAPBP-Interacting Protein  
Immunodeficiency due to Ficolin 3 Deficiency  
Immunodeficiency with Defective Leukocyte and Lymphocyte Function and with Response to Histamine-1 Antagonist 
Immunodeficiency without Anhidrotic Ectodermal Dysplasia  
IMMUNODEFICIENCY, DEVELOPMENTAL DELAY, AND HYPOHOMOCYSTEINEMIA  
Immunodeficiency, Gonadal Dysgenesis, and Pulmonary Fibrosis 
immunodeficiency-centromeric instability-facial anomalies syndrome +   
immunoglobulin alpha deficiency +   
immunoglobulin beta deficiency 
IMMUNOGLOBULIN KAPPA LIGHT CHAIN DEFICIENCY  
infectious mononucleosis  
Inosine Phosphorylase Deficiency, Immune Defect Due To 
INTELLECTUAL DEVELOPMENTAL DISORDER WITH SPEECH DELAY, DYSMORPHIC FACIES, AND T-CELL ABNORMALITIES  
Interleukin 2 Receptor, Alpha, Deficiency of  
Invasive Pneumococcal Disease, Recurrent Isolated, 1  
Invasive Pneumococcal Disease, Recurrent Isolated, 2  
IRAK4 Deficiency  
Kininogen Deficiency, High Molecular Weight and Low Molecular Weight 
Kotzot-Richter Syndrome 
lambda 5 deficiency 
Lichtenstein Syndrome 
LUNG DAMAGE, IMMUNODEFICIENCY AND CHROMOSOME BREAKAGE SYNDROME  
Lymphangiomyoma +   
Lymphoblastic Transformation, Intrinsic Defect in 
lymphocytic leukemia +   
Lymphoid System Deterioration, Progressive 
Lymphokine Deficiency 
lymphoma +   
lymphopenia +   
Lymphopenic Hypergammaglobulinemia, Antibody Deficiency, Autoimmune Hemolytic Anemia, and Glomerulonephritis 
lymphoproliferative syndrome +   
lymphoproliferative syndrome 1  
lymphoproliferative syndrome 2  
Lymphoproliferative Syndrome 3  
Macrophage Activation Syndrome  
Marek Disease 
MASP2 Deficiency  
multiple myeloma +   
MYD88 Deficiency  
Myelodysplasia, Immunodeficiency, Facial Dysmorphism, Short Stature, and Psychomotor Delay 
Natural Killer Cell and Glucocorticoid Deficiency with DNA Repair Defect  
NEMO Mutation with Immunodeficiency 
Neutrophil Immunodeficiency Syndrome  
Nezelof syndrome 
Paraproteinemias +   
Partial Combined Immunodeficiency with Absence of HLA Determinants and Beta-2-Microglobulin from Lymphocytes 
phagocyte bactericidal dysfunction +   
Plasma Clot Retraction Factor, Deficiency of 
plasmacytoma +   
Properdin Deficiency, X-Linked  
protein C deficiency +   
protein S deficiency +   
Riddle syndrome  
Roifman Syndrome  
Roifman-Chitayat Syndrome 
sarcoidosis +   
Schimke immuno-osseous dysplasia  
selective immunoglobulin deficiency disease +   
Sezary's disease  
sickle cell anemia +   
sideroblastic anemia with B-cell immunodeficiency, periodic fevers, and developmental delay  
Splenic Hypoplasia  
sulfhemoglobinemia +  
T cell deficiency +  
T Cell Immunodeficiency Primary  
T-Cell OKT4 Deficiency  
T-Cell Receptor-Alpha/Beta Deficiency  
Thumb Agenesis, Short Stature, and Immunodeficiency 
Tuftsin Deficiency 
Tumor Lysis Syndrome  
Waldenstroem's macroglobulinemia  
WHIM syndrome  
X-Linked Immunodeficiency with Deficiency of 115,000 Dalton Surface Glycoprotein 
X-linked immunodeficiency with magnesium defect, Epstein-Barr virus infection, and neoplasia  
X-linked lymphoproliferative syndrome 1  
X-linked lymphoproliferative syndrome 2  

Synonyms
Exact Synonyms: AGAMMAGLOBULINEMIA, AUTOSOMAL RECESSIVE, DUE TO IGHM DEFECT ;   Hypogammaglobulinemia ;   IGHM ;   agammaglobulinemias ;   hypogammaglobulinemias ;   mu heavy chain deficiency
Primary IDs: MESH:D000361
Alternate IDs: RDO:0000220
Xrefs: NCI:C26931 ;   OMIM:PS601495
Definition Sources: MESH:D000361, http://en.wikipedia.org/wiki/Dysgammaglobulinemia

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RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.