Diagnostic and therapeutic approaches for lymphoblastic lymphomas from progenitor cells in children and adolescents

Abstract

Lymphoblastic lymphomas (LBL) are the second most common subtype of non-Hodgkin’s lymphomas in children and adolescents, accounting for 25–35 % of all non-Hodgkin’s lymphomas cases. The majority of all lymphoblastic lymphomas (80–85 %) are T-lymphoblastic origin; 15–20 % origins from B-cell precursors, biphenotypic LBL are extremely rare. Currently, the overall and relapse-free survival of children and adolescents with LBL exceeds 80 %, uses of modern therapy programs. However, the survival rates of patients with recurrent or refractory LBL remain low – 10 %. Optimization of therapeutic approaches in LBL follows the path of clarifying clinical, morpho-immunological and molecular biological risk groups, modification of treatment programs using new drugs (including immunotherapy, inhibitors of multifunctional intracellular signaling pathways (NOTCH, PI3K/AKТ/mTOR, JAK/STAT and MAPK), affecting the cell cycle regulation), and also a reduction in immediate and long-term toxicity. This article presents modern approaches to the diagnosis of LBL, staging and choosing of a treatment method.