Lymphoblastic lymphoma in adults: diagnosis, prognosis and treatment

Lymphoblastic lymphoma is classified within the spectrum of acute lymphoblastic leukemia, though it possesses distinct clinical and biological characteristics that distinguish it from the latter. Prognostic factors are primarily based on genetic alterations associated with high-risk disease, as well as treatment response evaluated by PET-CT and measurable residual disease in the bone marrow. However, these data are derived from retrospective studies, and prospective validation is necessary. Treatment is based on protocols identical to those used for acute lymphoblastic leukemia, as they provide satisfactory and durable responses. Nevertheless, relapsed/refractory disease remains a challenge because most cases of lymphoblastic lymphoms are of T-cell lineage, for which the current therapeutic arsenal is very limited. The aim of this review is to describe the diagnosis, prognosis and therapeutic approaches in adult lymphoblastic lymphoma.