Indexed on: 25 Jan '17Published on: 25 Jan '17Published in: Blood
Angioimmunoblastic T-cell lymphoma (AITL) is an uncommon subtype of mature peripheral T-cell lymphoma (PTCL). The history of AITL is much longer and deeper than the literature would suggest given the many names that has preceded it. Advanced stage disease is common with uncharacteristic laboratory and autoimmune findings that often slow or mask the diagnosis. Significant strides in the immunohistochemical and molecular signature of AITL have brought increased ability to diagnose this uncommon type of PTCL. The 2016 World Health Organization (WHO) classification of lymphoid neoplasms recently acknowledged the complexity of this diagnosis with the addition of other AITL-like subsets. AITL now resides under the umbrella of nodal T-cell lymphomas with T Follicular helper phenotype. Induction strategies continue to focus on increasing complete remission rates that allow more transplant eligible patients to proceed toward consolidative high dose therapy and autologous stem cell rescue (HDT-ASCR) with improving long term survival. There are several clinical trials where recently approved drugs with known activity in AITL are paired with induction regimens with the hope to demonstrate long term progression free survival over CHOP. The treatment of relapsed or refractory AITL remains an unmet need. The spectrum of AITL from diagnosis to treatment is reviewed below in a fashion that may one day lead to personalized treatment approach in a many faced disease.