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Severe Combined Immunodeficiency (Bare Lymphocyte Syndrome)

Group of rare congenital disorders characterized by impairment of both humoral and cell-mediated immunity, leukopenia, and low or absent antibody levels. It is inherited as an X-linked or autosomal recessive defect. Mutations occurring in many different genes cause human Severe Combined Immunodeficiency (SCID).
Also Known As:
Bare Lymphocyte Syndrome; Immunodeficiency Syndrome, Severe Combined; Immunologic Deficiency, Severe Combined; Omenn's Syndrome; Reticuloendotheliosis, Familial; Severe Combined Immune Deficiency; Severe Combined Immunodeficiency Syndrome; Severe Combined Immunologic Deficiency; Bare Lymphocyte Syndromes; Combined Immunodeficiencies, Severe; Combined Immunodeficiency, Severe; Familial Reticuloendothelioses; Familial Reticuloendotheliosis; Immunodeficiencies, Severe Combined; Lymphocyte Syndrome, Bare; Lymphocyte Syndromes, Bare; Omenns Syndrome; Reticuloendothelioses, Familial; Severe Combined Immunodeficiencies; Syndrome, Bare Lymphocyte; Syndrome, Omenn; Syndrome, Omenn's; Syndromes, Bare Lymphocyte; Immunodeficiency, Severe Combined; Omenn Syndrome
Networked: 2749 relevant articles (108 outcomes, 202 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Neoplasms (Cancer)
2. Infections
3. Leukemia
4. Severe combined immunodeficiency due to adenosine deaminase deficiency
5. Melanoma (Melanoma, Malignant)

Experts

1. Kohn, Donald B: 35 articles (01/2022 - 10/2003)
2. Notarangelo, Luigi D: 35 articles (01/2022 - 02/2005)
3. Cowan, Morton J: 34 articles (01/2022 - 01/2008)
4. Puck, Jennifer M: 33 articles (01/2022 - 02/2002)
5. Aiuti, Alessandro: 31 articles (01/2021 - 06/2002)
6. Buckley, Rebecca H: 27 articles (01/2022 - 02/2002)
7. Gaspar, H Bobby: 25 articles (01/2022 - 10/2004)
8. Gennery, Andrew R: 24 articles (01/2022 - 09/2005)
9. Tateno, Chise: 22 articles (01/2018 - 09/2007)
10. Grunebaum, Eyal: 19 articles (01/2021 - 03/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Severe Combined Immunodeficiency:
1. Adenosine DeaminaseIBA
2. Proteins (Proteins, Gene)FDA Link
3. AntibodiesIBA
4. Immunotoxins (Immunotoxin)IBA
5. VaccinesIBA
6. Antiviral Agents (Antivirals)IBA
7. Urokinase-Type Plasminogen Activator (Urokinase)FDA Link
8. Immunoglobulins (Immunoglobulin)IBA
9. matrigelIBA
10. Cyclophosphamide (Cytoxan)FDA LinkGeneric

Therapies and Procedures

1. Transplantation
2. Hematopoietic Stem Cell Transplantation
3. Therapeutics
4. Bone Marrow Transplantation (Transplantation, Bone Marrow)
5. Cell Transplantation