A 32-year-old man presents with rapidly enlarging cervical lymphadenopathy, B symptoms and serum LDH 3x upper limit of normal. Biopsy shows large B-cells with prominent nucleoli and a 'starry-sky' pattern. FISH confirms MYC rearrangement at 8q24, BCL2 rearrangement at 18q21, and BCL6 rearrangement at 3q27. What is the WHO 2022 classification of this lymphoma?
- A Diffuse large B-cell lymphoma, NOS
- B High-grade B-cell lymphoma with MYC and BCL2 rearrangements (double-hit lymphoma)
- C High-grade B-cell lymphoma with MYC, BCL2 and BCL6 rearrangements (triple-hit lymphoma) ✓
- D Burkitt lymphoma with BCL2 co-rearrangement
Explanation
The WHO 2022 classification designates lymphomas with concurrent rearrangements of MYC plus BCL2 and/or BCL6 as 'high-grade B-cell lymphoma with MYC and BCL2/BCL6 rearrangements.' When all three genes are rearranged this is colloquially termed 'triple-hit lymphoma' and is categorised under the same WHO entity. These tumors have an extremely poor prognosis and require intensified regimens (e.g., DA-EPOCH-R) rather than standard R-CHOP. Burkitt lymphoma has MYC rearrangement alone and lacks BCL2/BCL6 co-rearrangement; DLBCL NOS lacks these defining cytogenetic findings.
Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.
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