Stratification and Treatment of Lymphoblastic Lymphoma

Lymphoblastic lymphoma (LBL) constitutes the second most common subtype of non-Hodgkin lymphoma in childhood and adolescence accounting for 25–30% of all cases. The majority of cases are of T-cell precursor origin (75%), while the remaining 25% of cases are of B-cell precursor origin. The development of a relapse-risk adapted acute lymphoblastic leukaemia-derived chemotherapy and implementation of the protocols in multinational therapy optimization trials allowed the treatment of paediatric LBL evolving successfully during the last 40 years with cure rates approaching 80–85%. In paediatric LBL, stage of disease, but also, recently, molecular genetics (T-LBL only) are used to determine composition and intensity of therapy. Still, 15–20% of patients progress or relapse, so that future therapy concepts aim at using conventional drugs more efficiently, but, in particular, at testing targeted drugs and cellular therapies directed towards lymphoma-associated alterations in order to improve cure rates and reduce therapy-associated acute and long-term toxicities.