Review
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World J Hematol. Nov 6, 2014; 3(4): 118-127
Published online Nov 6, 2014. doi: 10.5315/wjh.v3.i4.118
Follicular helper T lymphocytes in health and disease
Cecilia Parodi, María Noel Badano, Nora Galassi, Ana Coraglia, Patricia Baré, Alejandro Malbrán, María Marta de Elizalde de Bracco
Cecilia Parodi, Nora Galassi, Ana Coraglia, Patricia Baré, María Marta de Elizalde de Bracco, Department of Immunology, Instituto de Medicina Experimental (IMEX)– CONICET-Academia Nacional de Medicina (ANM) Pacheco de Melo 3081, Ciudad Autónoma de Buenos Aires 1425, Argentina
María Noel Badano, Patricia Baré, María Marta de Elizalde de Bracco, Department of Virology, Instituto de Investigaciones Hematológicas Mariano Castex (IIHEMA)-ANM, Pacheco de Melo 3081, Ciudad Autónoma de Buenos Aires 1425, Argentina
Alejandro Malbrán, Unidad de Alergia e Inmunología Clínica, Av. Pte. Roque Saenz Peña 1160, Ciudad Autónoma de Buenos Aires C1431GHA, Buenos Aires, Argentina
Author contributions: Parodi C, Badano MN, Galassi N and Coraglia A contributed equally to this work; Baré P participated in the discussion of TFH and viral diseases; Malbrán A participated in the discussion of TFH in the context of autoimmunity and immunodeficiency; de Elizalde de Bracco MM designed and wrote the manuscript.
Supported by Consejo Nacional de Investigaciones Científicas y Técnicas, CONICET, PIP Nos. 0032 and 11220120100619CO
Correspondence to: María Marta de Elizalde de Bracco, PhD, Chief, Department of Immunology, Instituto de Medicina Experimental (IMEX)-CONICET, Academia Nacional de Medicina (ANM), Pacheco de Melo 3081, Ciudad Autónoma de Buenos Aires 1425, Argentina. mebracco@hematologia.anm.edu.ar
Telephone: +54-11-48055759 Fax: +54-11-48039475
Received: May 7, 2014
Revised: July 12, 2014
Accepted: September 18, 2014
Published online: November 6, 2014
Processing time: 170 Days and 15.3 Hours
Abstract

A correct antibody response requires the participation of both B and T lymphocytes and antigen presenting cells. In this review we address the role of follicular helper T lymphocytes (TFH) in this reaction. We shall focus on the regulation of their development and function in health and disease. TFH can be characterized on the basis of their phenotype and the pattern of secretion of cytokines. This fact is useful to study their participation in the generation of antibody deficiency in primary immunodeficiency diseases such as common variable immunodeficiency, X-linked hyper IgM syndrome or X-linked lymphoproliferative disease. Increased numbers of TFH have been demonstrated in several autoimmune diseases and are thought to play a role in the development of autoantibodies. In chronic viral infections caused by the human immunodeficiency virus, hepatitis B or C virus, increased circulating TFH have been observed, but their role in the protective immune response to these agents is under discussion. Likewise, an important role of TFH in the control of some experimental protozoan infections has been proposed, and it will be important to assess their relevance in order to design effective vaccination strategies.

Keywords: Follicular helper T (TFH) lymphocytes; TFH development; Chemokine (C-X-C motif) receptor 5; Interleukin-21; Programmed cell death-1/Programmed cell death ligand 1 (PDL-1) or PDL-2; Primary immunodeficiencies; Autoimmunity; Chronic viral infections; Protozoan infections

Core tip: Follicular helper T lymphocytes (TFH) are essential to establish a correct and protective humoral immune response. Correct regulation of their development and differentiation is necessary to achieve a normal antibody response. They can be characterized by their phenotype and function. It has been proposed that their role is important in the generation of immunodeficiency or autoimmunity, as well as in the control of chronic viral or protozoan infections. This review comments recent advances in human TFH research that may be useful in order to design adequate therapeutic or vaccination strategies.