[MYC-associated B-cell lymphomas: pathophysiology and treatment]

Area of Science:

• Hematology
• Oncology
• Molecular Biology

Background:

• Double-hit lymphoma (DHL) is characterized by concurrent MYC and BCL2 and/or BCL6 rearrangements, leading to a poorer prognosis than diffuse large B-cell lymphoma (DLBCL).
• DHL patients typically exhibit resistance to R-CHOP therapy and aggressive disease progression, frequently involving extranodal sites and the central nervous system (CNS).
• "Double expressor status," defined by MYC and BCL2 protein co-expression, is a recognized poor prognostic biomarker in DLBCL, associated with an elevated risk of CNS relapse.

Purpose of the Study:

• To review the clinical and pathological features of MYC-associated B-cell lymphomas, including DHL and Burkitt lymphoma.
• To discuss the prognosis and current treatment strategies for DHL and double expressor lymphoma.
• To highlight new insights and the importance of CNS-directed evaluation and prophylaxis in high-risk patients.

Main Methods:

• Review of retrospective studies comparing chemotherapy regimens for DHL.
• Analysis of immunohistochemistry for MYC and BCL2 protein expression in DLBCL.
• Examination of clinical data regarding extranodal involvement and CNS relapse rates.

Main Results:

• Intensive chemotherapy regimens, such as dose-adjusted EPOCH-R, have shown more favorable outcomes in DHL compared to R-CHOP therapy in retrospective analyses.
• Double expressor status in DLBCL is linked to a higher risk of CNS relapse, underscoring the need for CNS-directed strategies.
• MYC-associated B-cell lymphomas, including DHL, present unique challenges in treatment and prognosis.

Conclusions:

• Patients with DHL and double expressor lymphoma require specialized treatment approaches beyond standard R-CHOP therapy.
• Intensive chemotherapy and proactive CNS prophylaxis are crucial for improving outcomes in these high-risk lymphoma subtypes.
• Further research into MYC-associated B-cell lymphomas is essential for developing more effective therapeutic strategies.