A 34-year-old woman presents with a rapidly enlarging neck mass. Biopsy shows large cells with voluminous pale cytoplasm in a 'starry sky' pattern, Ki-67 of nearly 100%, and positivity for CD10, BCL6, MYC, and BCL2 by IHC. FISH reveals concurrent translocations of MYC, BCL2, and BCL6. The WHO 2022 diagnosis is:
- A Burkitt lymphoma
- B High-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements (double/triple hit lymphoma) ✓
- C Diffuse large B-cell lymphoma, NOS
- D Primary mediastinal large B-cell lymphoma
Correct answer: B. High-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements (double/triple hit lymphoma)
Explanation
Concurrent rearrangements of MYC with BCL2 and/or BCL6 define 'double hit' (DH) or 'triple hit' (TH) lymphoma, classified by WHO 2022 as High-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements. These are aggressive and have poor prognosis with standard R-CHOP. Pure Burkitt lymphoma has MYC rearrangement alone and lacks BCL2 co-expression; DLBCL, NOS lacks confirmed DH/TH rearrangements.
Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.