Richter transformation in CLL refers to transformation into which histological subtype and is associated with which prognostic gene mutation?
- A Follicular lymphoma; BCL2 rearrangement
- B Mantle cell lymphoma; CCND1 translocation
- C Diffuse large B-cell lymphoma; TP53 mutation / NOTCH1 mutation ✓
- D Burkitt lymphoma; MYC translocation
Correct answer: C. Diffuse large B-cell lymphoma; TP53 mutation / NOTCH1 mutation
Explanation
Richter transformation (RT) occurs in 2–10% of CLL patients, most commonly transforming into DLBCL (>80% of cases) and rarely into Hodgkin lymphoma. TP53 mutation and NOTCH1 mutation are strongly associated with RT and confer a very poor prognosis (median survival <1 year). Clonal relationship between CLL and RT (>80% clonally related) worsens prognosis compared with clonally unrelated RT. The PET scan shows intense FDG uptake in transformed sites.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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