Major histocompatibility complex class II deficiency: Clinical manifestations, immunologic features, and outcome

The Journal of Pediatrics

Volumn 123, Issue 6, 1993, Pages 921-928

  Klein, Christoph ^a,b   Lisowska Grospierre, Barbara ^a,b   LeDeist, Françoise ^a,b   Fischer, Alain ^a,b   Griscelli, Claude ^a,b  

^a HÔPITAL NECKER ENFANTS MALADES   (France)

^b INSERM   (France)

Author keywords

[No Author keywords available]

Indexed keywords

ARTICLE; BONE MARROW TRANSPLANTATION; CAUSE OF DEATH; FATALITY; FEMALE; HUMAN; IMMUNE DEFICIENCY; MAJOR HISTOCOMPATIBILITY COMPLEX; MALE; ONSET AGE; PRIORITY JOURNAL; PROGNOSIS; T LYMPHOCYTE; VIRUS INFECTION;

CHILD; CHILD, PRESCHOOL; GENE EXPRESSION; GENES, MHC CLASS I; GENES, MHC CLASS II; HISTOCOMPATIBILITY ANTIGENS CLASS II; HUMAN; IMMUNOPHENOTYPING; INFANT; MAJOR HISTOCOMPATIBILITY COMPLEX; MALE; SEVERE COMBINED IMMUNODEFICIENCY; SUPPORT, NON-U.S. GOV'T;

EID: 0027446688     PISSN: 00223476     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0022-3476(05)80388-9     Document Type: Article

References (43)

1. Primary immunodeficiency diseases: report of a WHO scientific group
2. Impaired cell-to-cell interaction in partial immunodeficiency with variable expression of HLA antigens
3. Combined immunodeficiency with defective expression in MHC class II genes
4. Bone marrow transplantation in a patient with HLA defective combined immunodeficiency
5. Successful treatment with an unrelated-donor bone marrow transplant in an HLA-deficient patient with severe combined immunodeficiency (“bare lymphocyte syndrome”)
6. Failure of lymphocyte membrane HLA A and B expression in two siblings with combined immunodeficiency
7. Combined immunodeficiency disease associated with absence of cell surface HLA A and B antigen
8. A transacting class II regulatory gene unlinked to the MHC controls expression of HLA class II genes
9. Class II antigen negative patient and mutant B cell lines represent at least three and probably four distinct genetic defects defined by complementation analysis
10. The antibody spectrum in individuals with defective expression of HLA class II and the LFA-1 glycoprotein family genes
11. Microdroplet testing for HLA-A, -B, -C and -D antigens
12. Genotyping with DNA probes in combined immunodeficiency syndrome with defective expression of HLA
13. Age-related changes in human blood lymphocyte subpopulations
14. Combined immunodeficiency with defective expression of HLA: modulation of an abnormal HLA synthesis and functional studies
15. Immunodeficiency diseases and expression of HLA antigens
16. Clinical and immunological aspects of combined immunodeficiency with defective expression of HLA antigens
17. Clinical and immunological studies in patients with the “bare lymphocyte” syndrome
18. Congenital agammaglobulinemia associated with lack of expression of HLA-D-region antigens
19. The bare lymphocyte syndrome: report on the registry
20. “Bare lymphocytes” without immunodeficiency
21. Defective expression of HLA class I antigens: a case of the bare lymphocyte syndrome without immunodeficiency
22. Failure to thrive, thrush and hypogammaglobulinemia in a 6-month-old infant
23. Multiple and persistent viral infection in a patient with bare lymphocyte syndrome
24. Prenatal diagnosis of severe combined immunodeficiency with defective synthesis of HLA molecules
25. Congenital immunodeficiencies associated with absence of HLA class II antigens on lymphocytes result from distinct mutations in trans-acting factors
26. + T cells in major histocompatibility complex class II-deficient mice
27. Mice lacking MHC class II molecules
28. Severe combined immunodeficiency
29. Interferon-induced expression of class II major histocompatibility antigens in the major histocompatibility complex (MHC) class II deficiency syndrome
30. Regulation of genes for HLA class II antigens in cell lines from patients with severe combined immunodeficiency
31. Expression of MHC antigens in MHC class II deficiency
32. Interferonopathy in the bare lymphocyte syndrome
33. The thymus in “bare lymphocyte syndrome”: significance of expression of MHC antigens on thymic epithelial cells in intrathymic T cell maturation
34. Activation of genetically major histocompatibility complex (MHC) class II-deficient B lymphocytes
35. Signalling through the MHC class II cytoplasmic domain is required for antigen presentation and induces B7 expression
36. Congenital immunodeficiency with a regulatory defect in MHC class II gene expression lacks a specific HLA-DR promotor binding protein, RF-X
37. In vivo footprinting of MHC class II genes: bare promotors in the bare lymphocyte syndrome
38. Genetic complexity of regulatory mutants defective for HLA class II gene expression
39. Human chromosome 16 encodes a factor involved in induction of class II major histocompatibility antigens by interferon gamma
40. Recent advances in MHC class II deficiency
41. Bare lymphocyte syndrome: consequences of absent class II major histocompatibility antigen expression for B lymphocyte differentiation and function
42. European experience of bone-marrow transplantation for severe combined immunodeficiency
43. Reduction of graft failure by a monoclonal antibody (anti-LFA-1 CD11a) after HLA nonidentical bone marrow transplantation in children with immunodeficiencies, osteopetrosis, and Fanconi's anemia: a European Group for Immunodeficiency/European Group for Bone Marrow Transplantation report