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Term:
T-Cell Receptor-Alpha/Beta Deficiency (DOID:9000611)
Annotations: Rat: (0) Mouse: (1) Human: (2) Chinchilla: (0) Bonobo: (0) Dog: (1) Squirrel: (0) Pig: (1)
Parent Terms Term With Siblings Child Terms
Abnormal Neutrophil Chemotactic Response 
acidophil adenoma 
agammaglobulinemia +   
aggressive NK-cell leukemia 
Anhidrotic Ectodermal Dysplasia with Immunodeficiency, Osteopetrosis, and Lymphedema  
Anhidrotic Ectodermal Dysplasia with T-Cell Immunodeficiency, Autosomal Dominant  
Antibody Deficiency due to Defect in CD19 
ataxia telangiectasia +   
Autoinflammation, Antibody Deficiency, and Immune Dysregulation, PLCG2-Associated  
autosomal dominant familial periodic fever  
B cell deficiency +   
B-Cell Immunodeficiency, Distal Limb Anomalies, and Urogenital Malformations 
B-cell lymphoma +   
basophil adenoma 
basophilic adenocarcinoma 
C1q Deficiency  
C9 Deficiency with Dermatomyositis 
CARD11 Immunodeficiency  
Cartilage Hair Hypoplasia Like Syndrome 
cartilage-hair hypoplasia  
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 deficiency +   
Congenital Erythroderma with Palmoplantar Keratoderma, Hypotrichosis, and Hyper-IgE  
Davenport Donlan Syndrome 
Deltaretrovirus Infections +   
dendritic cell sarcoma +  
dendritic cell thymoma +  
Endotoxin Hyporesponsiveness  
Eosinophilia +   
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 
Griscelli syndrome +   
Hepatic Venoocclusive Disease with Immunodeficiency  
hereditary neutrophilia  
human immunodeficiency virus infectious disease +   
Hypoglobulinemia and Absent B Cells 
IL21R Immunodeficiency  
Immune Deficiency Disease  
Immune Deficiency, Familial Variable 
IMMUNODEFICIENCY 11B WITH ATOPIC DERMATITIS  
Immunodeficiency 12  
Immunodeficiency 14  
IMMUNODEFICIENCY 15 +   
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 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  
Kotzot-Richter Syndrome 
Lazy Leukocyte Syndrome 
leukocyte adhesion deficiency +   
Leukocyte Nuclear Appendages, Hereditary Prevalence of 
Leukocytosis +   
leukopenia +   
leukostasis  
Lichtenstein Syndrome 
LUNG DAMAGE, IMMUNODEFICIENCY AND CHROMOSOME BREAKAGE SYNDROME  
Lymphoblastic Transformation, Intrinsic Defect in 
Lymphoid System Deterioration, Progressive 
Lymphokine Deficiency 
lymphopenia +   
Lymphopenic Hypergammaglobulinemia, Antibody Deficiency, Autoimmune Hemolytic Anemia, and Glomerulonephritis 
lymphoproliferative syndrome +   
MASP2 Deficiency  
mastocytosis +   
Monocyte Esterase Deficiency  
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 Actin Dysfunction 
Neutrophil Immunodeficiency Syndrome  
Nezelof syndrome 
Nisch syndrome  
Partial Combined Immunodeficiency with Absence of HLA Determinants and Beta-2-Microglobulin from Lymphocytes 
Pelger-Huet anomaly +   
Pelger-Huet-Like Anomaly and Episodic Fever with Abdominal Pain 
phagocyte bactericidal dysfunction +   
Phagocytosis, Plasma-Related Defect in 
Presentey Anomaly  
Properdin Deficiency, X-Linked  
Riddle syndrome  
Roifman Syndrome  
Roifman-Chitayat Syndrome 
Schimke immuno-osseous dysplasia  
sideroblastic anemia with B-cell immunodeficiency, periodic fevers, and developmental delay  
Specific Granule Deficiency +   
Splenic Hypoplasia  
T cell deficiency +  
T Cell Immunodeficiency Primary  
T-cell leukemia +   
T-Cell OKT4 Deficiency  
T-Cell Receptor-Alpha/Beta Deficiency  
Thumb Agenesis, Short Stature, and Immunodeficiency 
Tuftsin Deficiency 
Undritz Anomaly 
WHIM syndrome  
X-Linked Immunodeficiency with Deficiency of 115,000 Dalton Surface Glycoprotein 

Synonyms
Exact Synonyms: IMD7 ;   IMMUNODEFICIENCY 7 ;   TCR-Alpha/Beta Deficiency
Primary IDs: OMIM:615387 ;   RDO:9000928

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