Transformed follicular lymphoma

Abstract

Follicular Lymphoma (FL) is the second most common type of non-Hodgkin lymphoma and is considered to be the prototype of indolent lymphomas. Histologic transformation into an aggressive lymphoma, which is expected to occur at a rate of 2 to 3% each year, is associated with rapid progression, treatment resistance, and poor prognosis. Recent modifications to the physiopathologic mechanism of transformed follicular lymphoma (t-FL) have been proposed, including genetic and epigenetic mechanisms as well as a role for the microenvironment. Although t-FL is considered a devastating complication, as it is associated with treatment-refractory disease and a dismal outcome, recent data in the rituximab era have suggested that not only is the prognosis less severe than reported in the previous literature but the risk of transformation is also lower. Thus, this study aimed to review the most recent research on t-FL in an attempt to better understand the clinical meaning of transformation from FL to diffuse large B cell lymphoma (DLBCL) and the impact of current treatment strategies on the curability of this intriguing subentity of lymphoma.

A review of chimeric antigen receptor T-cells in lymphoma

“…Despite improvement in survival in FL and tFL in the Rituximab era, refractory disease remains difficult to control and patients previously exposed to anthracycline based therapy and those not eligible for SCT have few treatment options available to them making the advent of novel therapies critical for this group [35]. Axicabtagene ciloleucel has been approved for use in refractory tFL based on results from the ZUMA-1 trial which shows 83% ORR and 63% CR in patients with tFL and PMBCL [14].…”

Transformed follicular lymphoma in the rituximab era: A report from the Spanish Lymphoma Oncology Group

“…The histological transformation (HT) of FL is an event considered frequent in the natural history of this tumor. FL is usually transformed into diffuse large B‐cell lymphoma (DLBCL), lymphoma/lymphoblastic leukemia (ALL), and others such as the recently named “unclassifiable B lymphomas.”…”

“…FL is usually transformed into diffuse large B-cell lymphoma (DLBCL), 1,2 lymphoma/lymphoblastic leukemia (ALL), 3,4 and others such as the recently named "unclassifiable B lymphomas." 5 The current rate of HT stands at 10%.…”

Lymphoma 2020: an update on recent advances in pathobiology and application to clinical practice

“…10 Additional genetic events may also lead to transformation to diffuse large Bcell lymphoma that occur at a rate of 2-3% of patients per year following the diagnosis of FL. 11 Randall and Fedoriw also review several types of extranodal FL. These neoplasms are often negative for t(14;18)(q32;q21)/IGH-BCL2 or have other unusual features and include primary cutaneous follicle centre cell lymphoma, paediatric-type FL, FL associated with IRF4 rearrangement, testicular FL, and duodenal-type FL.…”