Molecular etiology of mature T-cell non-hodgkins lymphomas

摘要: T-cell non-Hodgkin's lymphomas (NHL) represent approximately 10-15% of all diagnosed in Western countries. Significant progress has been made over the last 2 decades defining non-random chromosomal abnormalities. Cytogenetic and molecular analyses have enhanced diagnostic capabilities as well improved classification prognostication for NHL. Gamma-delta receptor (TCR) clonality now represents more common TCR rearrangement subcutaneous panniculitis-like lymphoma (SCPTCL), hepatosplenic (HSTCL), extranodal NK/T-cell lymphoma, nasal type enteropathy-type intestinal (EITCL). Non-random, recurrent chromosome abnormalities such isochrome 7 with HSTCL, complex karyotypes peripheral not otherwise characterized (PTCL-NOC), trisomies 3 5 angioimmunoblastic (AIL) t(2;5) systemic anaplastic recognized. Furthermore, identification relevant protooncogenes tumor suppressor genes involved pathogenesis NHL NPM/ALK fusion protein, p53, cyclin dependent kinase inhibitors (p15, p16, p21) EBV their interplay various regulatory pathways cell cycle progression apoptosis potential candidates molecular-based therapy. This review presents a detailed analysis genetic perturbations present mature including discussion how tumor-specific alterations impact on clinical outcome. Future studies are likely to provide additional disease-specific translocations important translational implications.