CD40 Ligand Deficiency
Allergol. immunopatol
; 48(4): 409-413, jul.-ago. 2020.
Artigo
em Inglês
| IBECS
| ID: ibc-199728
Biblioteca responsável:
ES1.1
Localização: BNCS
ABSTRACT
CD40 ligand deficiency (CD40L), currently classified as an inborn error of immunity affecting cellular and humoral immunity, prevalently emerges in boys within the first two years of life. It manifests itself as a decrease in serum IgG, IgA and IgE, with normal or high IgM, defects in T cell proliferation, and decrease in soluble CD40L. These accompany sinopulmonary and/or gastrointestinal infections, and there may be infections caused by pyogenic bacteria, opportunistic infections, autoimmune diseases, and neoplasms. Mild and moderate cases of this deficiency may respond well to prophylactic antibiotic therapy or to human immunoglobulin replacement therapy, in addition to the early treatment of infections. Severe cases can be treated with hematopoietic stem cell transplantation, which allows the healing of such patients, rather than sequelae and a poor progression. Thus, its differential diagnosis with other inborn errors of immunity is essential, especially CD40 deficiency and variable common immunodeficiency; the reason why we have proposed the present literature review
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Bases de dados nacionais
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Espanha
Base de dados:
IBECS
Assunto principal:
Ligante de CD40
/
Síndrome de Imunodeficiência com Hiper-IgM Tipo 1
/
Doenças do Sistema Imunitário
Limite:
Humanos
Idioma:
Inglês
Revista:
Allergol. immunopatol
Ano de publicação:
2020
Tipo de documento:
Artigo
Instituição/País de afiliação:
Irmandade da Santa Casa de Misericódia de São Paulo/Brazil