Study of adaptative immunity for the production of antibodies to polysaccharide antigens in patients with primary immunodeficiency

• Grant number: 15/20726-1
• Support Opportunities: Regular Research Grants
• Start date: September 01, 2016
• End date: August 31, 2019
• Field of knowledge: Health Sciences - Medicine
• Principal Investigator: Maria Isabel de Moraes Pinto
• Grantee: Maria Isabel de Moraes Pinto
• Host Institution: Escola Paulista de Medicina (EPM). Universidade Federal de São Paulo (UNIFESP). Campus São Paulo. São Paulo , SP, Brazil
• Associated researchers: Daniélli Christinni Bichuete Silva ; Fernanda Aimée Nobre ; Fernanda Cabral Cardoso Hardt ; Isabela Garrido da Silva Gonzalez ; Juliana Themudo Lessa Mazzucchelli ; Maria Isabel de Moraes Pinto ; Mariana de Gouveia Pereira ; Milena Karina Coló Brunialti ; Reinaldo Salomão

Abstract

The inability to produce antibodies to polysaccharide antigens (SAD-specific antibody deficiency) can present as a Primary Immunodeficiencies (IDP) specify or associated with other IDP such as ataxia-telangiectasia (AT) and Wiskott-Aldrich syndrome (WAS).The response to polysaccharide antigens, considered a T-independent response, is carried by B cells of the marginal zone of the spleen, B-1 cells present in the peritoneal cavity and mucous membranes. It has assigned importance to peripheral blood cells CD27 + IgM + B cells or IgM memory that recirculate in the marginal zone of the spleen. Innate immunity has been increasingly implicated in the targeting of the adaptive response once the cell interaction with bacteria and their products are recognized by pattern recognition receptors (PRR), which CD14 and "Toll-like-receptors" (TLRs ) 2 and 4 induce IL-6 and TNF-a secretion .GoalsTo study the innate and adaptive response to pneumococcus in patients with SAD, AT and WAS:1. To study B lymphocytes subsets (IgM memory, B1, CD21, with or without memory class switch) in patients with SAD WAS and AT.2. To study the follicular T cells in patients with SAD, WAS and AT.3. To study the follicular T cells in patients with Agammaglobulinemia4. To compare results to ICV patients and matched control group.CasuistPatients of both sex, aged between 4 and 30 years treated at the Clinical Immunology sector and Inborn Errors of Metabolism UNIFESP-EPM. PID diagnosis will be according to the ESID (www.esid.org) criteria. SAD: n = 15, Ataxia Telangiectasia-n = 15, Wiskott-Aldrich: n = 8, ICV: n = 15 controls: n = 15. All patients should be without acute infection at the time of blood collection. An appropriate response after immunization will be considered when the antibody concentration is greater than or equal to 1.3 mcg / ml. (AU)