A 50-year-old man with CLL on ibrutinib develops progressive lymphocytosis with rapid lymphadenopathy. Biopsy of the lymph node shows sheets of large centroblast-like cells with high Ki-67 (>80%), strong CD20, and MYC rearrangement. This complication is known as:
- A Accelerated phase CLL with prolymphocytoid transformation
- B Follicular lymphoma transformation to high-grade B-cell lymphoma
- C Clonal evolution with del(17p) without histologic transformation
- D Richter transformation to diffuse large B-cell lymphoma ✓
Explanation
Richter syndrome (transformation) refers to the development of aggressive lymphoma, most commonly DLBCL, in patients with CLL/SLL. It occurs in 5–10% of CLL patients, carries a poor prognosis (median survival 8–12 months), and is associated with MYC rearrangement, TP53 mutation, NOTCH1 mutation, and CDKN2A deletion. The transformation may be clonally related or unrelated to the original CLL clone. Accelerated-phase CLL involves increased prolymphocytes (>55%) but retains small lymphocyte architecture. Follicular lymphoma transforms to DLBCL or high-grade B-cell lymphoma but in a different clinical context. del(17p) represents molecular progression without histologic transformation.
Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.
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