Transcriptomic Abnormalities in Epstein Barr Virus Associated T/NK Lymphoproliferative Disorders.

Area of Science:

• Hematology
• Oncology
• Molecular Biology

Background:

• Epstein Barr virus-positive T/NK lymphoproliferative disorders (EBV-TNKLPD) encompass a range of neoplasms, including extranodal NK/T-cell lymphoma (ENKTL).
• The molecular biology of pediatric EBV-TNKLPD remains poorly understood.
• RNA abnormalities significantly influence the biology, classification, and treatment of EBV-TNKLPD.

Purpose of the Study:

• To review key transcriptomic aberrancies in EBV-TNKLPD.
• To discuss the translational potential of these findings for novel therapies.

Main Methods:

• Gene and miRNA expression profiling.
• Analysis of oncogenic signaling pathways.
• Review of current literature on EBV-TNKLPD.

Main Results:

• Upregulation of proliferation pathways (JAK/STAT, NF-kB) in ENKTL.
• Inhibition of apoptosis by survivin and p53 deregulation in ENKTL and chronic active EBV infection (CAEBV).
• Immune evasion mediated by the PD-1/PD-L1 pathway in ENKTL.
• Identification of EBV-positive PTCL-NOS with a distinct molecular signature.

Conclusions:

• Transcriptomic profiling is crucial for classifying EBV-TNKLPD and identifying novel therapeutic targets.
• Immune checkpoint inhibition and JAK inhibition show promise for treating EBV-TNKLPD, with ongoing clinical trials.