Bare lymphocyte syndrome 2

Also Known As:

BLS Type II; Bare Lymphocyte Syndrome, Type II; Bare lymphocyte syndrome type 2; SCID, HLA Class 2-Negative; SCID, HLA Class II-Negative; Severe combined immunodeficiency, HLA class ii-negative

Networked: 4 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Inborn Genetic Diseases: 11939
  - Primary Immunodeficiency Diseases: 1189
    - Severe Combined Immunodeficiency: 2749
      - Bare lymphocyte syndrome 2: 4
- Diseases Newborn Infant: 266
  - Severe Combined Immunodeficiency: 2749
    - Bare lymphocyte syndrome 2: 4
Nutritional and Metabolic Diseases: 15
- Metabolic Diseases: 10142
  - DNA Repair-Deficiency Disorders: 538
    - Severe Combined Immunodeficiency: 2749
      - Bare lymphocyte syndrome 2: 4
Immune System Diseases: 4321
- Immunologic Deficiency Syndromes: 91
  - Primary Immunodeficiency Diseases: 1189
    - Severe Combined Immunodeficiency: 2749
      - Bare lymphocyte syndrome 2: 4

Related Diseases

1.	Infections
2.	Severe Combined Immunodeficiency (Bare Lymphocyte Syndrome)
3.	Primary Immunodeficiency Diseases

Experts

1.	Bredius, Robbert: 1 article (01/2022)
2.	Damoiseaux, Jan: 1 article (01/2022)
3.	Damoiseaux, Maurits: 1 article (01/2022)
4.	Pico-Knijnenburg, Ingrid: 1 article (01/2022)
5.	van Well, Gijs: 1 article (01/2022)
6.	van der Burg, Mirjam: 1 article (01/2022)
7.	Abolnezhadian, Farhad: 1 article (01/2020)
8.	Dehghani, Razieh: 1 article (01/2020)
9.	Dehnavi, Sajad: 1 article (01/2020)
10.	Khodadadi, Ali: 1 article (01/2020)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Bare lymphocyte syndrome 2:

1.	T-Cell Antigen Receptors (T-Cell Receptor)IBA 01/01/2022 - "Genetic analysis revealed a novel RFXANK mutation, c.232C > T, resulting in a stop codon, with consequently defective transcription of MHC class II resulting in bare lymphocyte syndrome (BLS) type II. The initial unawareness of complete absence of MHC class II expression and normal T-cell receptor excision circles (TREC)-levels delayed the final diagnosis. "
2.	HLA Antigens (Human Leukocyte Antigens)IBA 03/17/1984 - "The Bare Lymphocyte Syndrome type II consists of lack of expression of class II HLA antigens, associated with immunodeficiency, and the postulated type III consists of complete lack of any class I and II HLA antigen at cell surface. "
3.	Codon (Codons)IBA 01/01/2022 - "Genetic analysis revealed a novel RFXANK mutation, c.232C > T, resulting in a stop codon, with consequently defective transcription of MHC class II resulting in bare lymphocyte syndrome (BLS) type II. The initial unawareness of complete absence of MHC class II expression and normal T-cell receptor excision circles (TREC)-levels delayed the final diagnosis. "
4.	Histocompatibility Antigens Class IIIBA 01/01/2020 - "Recurrence of severe microbial infections results from a primary immunodeficiency disorder known as major histocompatibility complex class II deficiency or bare lymphocyte syndrome type II. Immunologic function is severely impaired due to the absence of MHC class II molecules on the surface of immune cells. "
5.	AntigensIBA 03/17/1984 - "The Bare Lymphocyte Syndrome type II consists of lack of expression of class II HLA antigens, associated with immunodeficiency, and the postulated type III consists of complete lack of any class I and II HLA antigen at cell surface. "

Therapies and Procedures

1.	Hematopoietic Stem Cell Transplantation 10/01/2014 - "We describe the first case, to our knowledge, of disseminated Mycobacterium bovis Bacillus Calmette-Guérin infection in a child with Bare Lymphocyte Syndrome type II after undergoing hematopoietic stem cell transplantation (HSCT). "