Anaplastic large cell lymphoma

Abstract

Anaplastic large cell lymphoma (ALCL) is a rare lymphoid malignancy that is characterized by the expression of CD30 cell surface receptor and by a chromosomal translocation that results in aberrant constitutive activation of the anaplastic large cell lymphoma kinase (ALK). Like many other types of lymphoma, no major advances in the treatment of ALCL have been made for almost three decades. This is mainly because the relatively high cure rate and the rarity of the disease. Current frontline multi-agent chemotherapy regimens cure approximately 50 % of the patients, and patients with ALK-positive lymphoma usually have a better prognosis. More recently, advances in the molecular biology of ALCL have led to the development of new targeted agents. Brentuximab vedotin which targets CD30 receptor induces 85 % response rate in patients with relapsed ALCL and was recently approved by the US Food and Drug Administration for this disease. Crizotinib, which targets the ALK kinase and has shown promising results in preclinical and a limited clinical setting, is currently being evaluated in phase II clinical studies. Future directions will aim at changing current treatment strategies by incorporating these two new agents in frontline regimens.