A 25-year-old presents with lymphadenopathy and a biopsy showing a centroblastic tumor with a germinal-center immunophenotype (CD10+, BCL-6+, MUM1-). The Ki-67 is 95%, and fluorescence in situ hybridization (FISH) shows rearrangements of MYC and BCL-2. Which diagnosis is correct and what is the clinical implication?

• A Burkitt lymphoma; treat with intensive short-cycle chemotherapy
• B Double-hit lymphoma (DHL); highly aggressive, requires intensive regimens beyond R-CHOP ✓
• C Primary mediastinal large B-cell lymphoma; treat with DA-EPOCH-R
• D Follicular lymphoma Grade 3B transformed; treat with R-CHOP

Explanation

Concurrent MYC and BCL-2 rearrangements define a 'double-hit' high-grade B-cell lymphoma (HGBL-DH), now classified separately in WHO 2022 as HGBL with MYC and BCL-2 rearrangements. The germinal-center phenotype (CD10+, BCL-6+, MUM1-) is characteristic. DHL is highly aggressive with poor response to standard R-CHOP, requiring intensified regimens such as DA-EPOCH-R or R-CODOX-M/IVAC. Burkitt lymphoma has t(8;14) with MYC rearrangement alone and extremely high Ki-67 (approaching 100%) with a starry-sky pattern.

Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.