Introduction: Angioimmunoblastic T-cell lymphoma (AITL) is a type of peripheral T-cell lymphomas harboring aggressive biological behaviors and presenting unique immunologic hyperactivation. Gene expression profiling has revealed that AITL cells derive from follicular T helper (Tfh) cells. The prognosis is poor under CHOP-like regimen, and salvage chemotherapy is mostly ineffective. Thus, novel therapies are required in patients with AITL. NCCN Guidelines for peripheral T-cell lymphoma refer to cyclosporin (CyA) as one therapeutic option in second- or later lines especially for AITL cases unfeasible for hematopoietic stem-cell transplantation (HSCT). The clinical data of AITL are based on small-scale case series, and prospective trials are scarce.Areas covered: In this article, the authors reviewed published articles or conference abstracts, and extracted total 26 patients with AITL receiving CyA.Expert opinion: The overall response rate in above 26 patients was outstanding (86% in second or later lines), suggesting the utility in heavily treated cases including those who underwent HSCT. Meanwhile, publication bias is the most serious limitation in this literature review. Larger case series or prospective data with CyA alone or in combination with other drugs are warranted to establish the position in the treatment strategy of patients with AITL.
Keywords: Angioimmunoblastic T-cell lymphoma; cyclosporin; follicular T helper cells; novel agents; salvage chemotherapy.