Summary:
Follicular center helper T-cells (T-fh cells) are a subset of CD4+ T-cell
which are linked to several human autoimmune conditions, primary
immunodeficiency and cancer. T-fh cells characteristically produce IL21 and IL4
and show high expression of surface markers such us CXCR5, PD1, ICOS and the
chemokine CXCL13. Their role is pivotal in the activation of B-cells in the
germinal centers of secondary lymphoid organs.
Many studies have demonstrated that T-fh are the cells of origin of specific
lymphoma subtypes : AITL, 20-30% of PTCL-NOS, F-PTCL, Mycosis Fungoids, Sezary
Syndrome and primary cutaneous CD4+ small/medium-sized T-cell lymphoma.
The understanding of T-fh cell intracellular pathways, receptors,
transcription factors and chemokines/cytokines offers possible treatment by
targeting the malignant T-fh cells.
Moreover, autoimmune disorders related to Τ-FH could be repaired by targeted
new therapies.
Keywords:
Τ lymphoma, T follicular helper cells, Angioimmunoblastic lymphoma, Mycosis fungoides/Syndrome Sezary, Peripheral T-cell lymphoma (PTCL-NOS)
