Emerging Therapeutic Landscape of Peripheral T-Cell Lymphomas Based on Advances in Biology: Current Status and Future Directions

Simple Summary: Peripheral T-cell lymphoma is a rare but aggressive tumor. Due to its rarity, the disease has not been completely understood. In our review, we look at this lymphoma at the molecular level based on available literature. We highlight the mechanism behind the progression and resistance of this tumor. In doing so, we bring forth possible mechanism that could be exploited through novel chemotherapy drugs. In addition, we also look at the current available drugs used in treating this disease, as well as highlight other new drugs, describing their potential in treating this lymphoma. We comprehensively have collected and present the available biology behind peripheral T-cell lymphoma and discuss the available treatment options. T-cell lymphomas are a relatively rare group of malignancies with a diverse range of pathologic features and clinical behaviors. Recent molecular studies have revealed a wide array of different mechanisms that drive the development of these malignancies and may be associated with resistance to therapies. Although widely accepted chemotherapeutic agents and combinations, including stem cell transplantation, obtain responses as initial therapy for these diseases, most patients will develop a relapse, and the median survival is only 5 years. Most patients with relapsed disease succumb within 2 to 3 years. Since 2006, the USFDA has approved five medications for treatment of these diseases, and only anti-CD30-therapy has made a change in these statistics. Clearly, newer agents are needed for treatment of these disorders, and investigators have proposed studies that evaluate agents that target these malignancies and the microenvironment depending upon the molecular mechanisms thought to underlie their pathogenesis. In this review, we discuss the currently known molecular mechanisms driving the development and persistence of these cancers and discuss novel targets for therapy of these diseases and agents that may improve outcomes for these patients.