Recent Advances in Diagnosis and Therapy of Angioimmunoblastic T Cell Lymphoma

Abstract

Angioimmunoblastic T cell lymphoma (AITL) is a common subtype of mature peripheral T cell lymphoma (PTCL). As per the 2016 World Health Organization classification, AITL is now considered as a subtype of nodal T cell lymphoma with follicular helper T cells. The diagnosis is challenging and requires a constellation of clinical, laboratory and histopathological findings. Significant progress in the molecular pathophysiology of AITL has been achieved in the past two decades. Characteristic genomic features have been recognized that could provide a potential platform for better diagnosis and future prognostic models. Frontline therapy for AITL was mainly depending on chemotherapy and the management of relapsed or refractory AITL is still unsatisfactory with a very poor prognosis. Upfront transplantation offers better survival. Novel agents have been introduced recently with promising outcomes. Several clinical trials of combinations using novel agents are underway. Herein, we briefly review recent advances in AITL diagnosis and the evolving treatment landscape.

Relapsed Angioimmunoblastic T Cell Lymphoma with Fulminant Leukemic Involvement

“…Management of patients with relapse/refractory (R/R) disease should include careful assessment of the patient’s eligibility for autologous stem cell transplantation (ASCT) [ 18 ], although this can be very challenging given the disease-related symptoms and comorbidities that most patients have [ 14 , 19 ]. Delivery of high-dose therapy (HDT) followed by ASCT (HDT-ASCT) is considered the standard of care for R/R lymphomas, including PTCL.…”

“…Delivery of high-dose therapy (HDT) followed by ASCT (HDT-ASCT) is considered the standard of care for R/R lymphomas, including PTCL. However, results are dismal in PTCL patients, with a relapse rate over 80% [ 18 , 20 ]. A multicenter study that evaluated long-term disease-free survival in 29 patients with AITL after HDT-ASCT found that 5-year overall and event-free survival were 44% and 37%, respectively [ 21 ].…”

“…The addition of monoclonal antibodies directed against some of the antigens expressed by neoplastic lymphoma cells showed an impressive improvement in B cell lymphoma [ 18 ]. Brentuximab vedotin (BV) is an anti-CD30 monoclonal antibody-drug conjugate, and since CD30 is expressed in most AITL [ 25 ], BV has been investigated as a promising agent [ 18 , 26 ].…”

“…The addition of monoclonal antibodies directed against some of the antigens expressed by neoplastic lymphoma cells showed an impressive improvement in B cell lymphoma [ 18 ]. Brentuximab vedotin (BV) is an anti-CD30 monoclonal antibody-drug conjugate, and since CD30 is expressed in most AITL [ 25 ], BV has been investigated as a promising agent [ 18 , 26 ]. A significant improvement in PFS (48.2 months) was seen in patients treated with BV combined with CHP compared to CHOP for CD30-positive PTCL in the ECHELON-2 study [ 18 , 27 ].…”

Recurrence of Angioimmunoblastic T-cell Lymphoma in a Patient Successfully Treated for Hodgkin’s Lymphoma: A Case Report

“…Later, it was recognized as angioimmunoblastic T-cell lymphoma (AITL) in the revised European-American classification of lymphoid neoplasms (REAL) and World Health Organization (WHO) classifications [ 1 ]. It is a rare disease and accounts for approximately 15%-30% of peripheral nodal T-cell lymphomas (PTCL) globally and represents 2% of all non-Hodgkin’s lymphomas (NHLs) [ 1 , 2 ]. No consistent risk factors or etiological agents have been identified in AITL [ 1 ]; however, there is some association with Epstein-Barr virus (EBV) and several reports have shown that high EBV viremia upon presentation was associated with a worse response, disease progression, or evolution into aggressive B-cell lymphoma [ 2 ].…”