An update in treating transformed lymphoma

Indolent lymphomas typically have a prolonged course and favorable prognosis. Recent data support survival times that can reach several decades, even if periodic treatment is needed to manage symptoms or stabilize disease. However, all indolent lymphomas have the potential to undergo transformation to an aggressive phenotype, clinically characterized by a rapid progression of adenopathy, new-onset constitutional symptoms, or laboratory abnormalities, and the immediate need for therapeutic intervention. The most common scenario is transformation of follicular lymphoma to either diffuse large B-cell lymphoma or high-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 translocations; however, other indolent subtypes such as marginal zone lymphoma, lymphoplasmacytic lymphoma, small lymphocytic lymphoma/chronic lymphocytic leukemia, or even nodular lymphocyte predominant Hodgkin lymphoma, can undergo similar histologic transformation. The prognosis of transformed lymphomas has historically been quite poor, but there is ample evidence this is changing in the rituximab era. This article will provide a review of transformed lymphomas with an emphasis on treatment and the nuances of diagnosis and clinical management. Unless otherwise specified, all discussion in this review pertains to transformed follicular lymphoma which is the more common scenario and the subtype with the most robust data. In many cases, this information can be extrapolated and applied to other indolent histologies (i.e. transformed marginal zone lymphoma); however, several other clinical scenarios, such as Richter's transformation and “double hit” transformations, warrant a distinct discussion and will be reviewed separately.