A 58-year-old man presents with painless cervical lymphadenopathy. Biopsy shows diffuse proliferation of large B cells with CD20+, CD10+, BCL6+, MYC+ expression, and Ki-67 of 90%. FISH reveals MYC rearrangement AND BCL2 rearrangement. The specific diagnosis and prognosis is:
- A Double-hit lymphoma (DHL); poor prognosis requiring intensified therapy beyond standard R-CHOP ✓
- B Burkitt lymphoma; excellent prognosis with standard chemotherapy
- C Follicular lymphoma Grade 3B; indolent course
- D Mantle cell lymphoma; treat with ibrutinib
Explanation
Concurrent MYC rearrangement with BCL2 (and/or BCL6) rearrangement defines double-hit (or triple-hit) high-grade B-cell lymphoma, which carries a very poor prognosis with standard R-CHOP. These lymphomas are classified as High-Grade B-Cell Lymphoma with MYC and BCL2/BCL6 rearrangements in the WHO 5th edition and require intensified induction (e.g., R-DA-EPOCH or R-HyperCVAD) with consideration of consolidative autologous SCT. Burkitt lymphoma has a single MYC rearrangement with unique morphology. Follicular and mantle cell lymphomas have different immunophenotypes and genetic profiles.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.