The 2022 WHO classification of haematolymphoid tumours introduced 'large B-cell lymphoma with IRF4 rearrangement' as a distinct entity. Which of the following best characterises this entity?
- A Occurs predominantly in children/young adults, involves Waldeyer ring, usually MYC-negative, carries favourable prognosis ✓
- B Arises exclusively in elderly immunosuppressed patients, shows EBV positivity in >90% of cases
- C Characterised by BCL2 and MYC dual rearrangement ('double-hit'), classified as high-grade B-cell lymphoma
- D Is a variant of follicular lymphoma arising in bone marrow with paraprotein production
Explanation
Large B-cell lymphoma with IRF4 rearrangement is recognised as a distinct entity in the WHO 2022 classification (also reflected in the ICC 2022). It predominantly affects children and young adults (median age ~20 years), arises in the Waldeyer ring (tonsils, adenoids) and cervical lymph nodes, shows strong MUM1/IRF4 protein expression, and is characterised by IGH-IRF4 or other IRF4 translocations. Notably, despite morphological overlap with DLBCL and FL grade 3B, it lacks MYC, BCL2, and BCL6 rearrangements and carries an excellent prognosis with standard R-CHOP. It should not be classified as double-hit lymphoma.
Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.
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