Richter's transformation in CLL is best characterised by:
- A Transformation to acute myeloid leukaemia with poor response to cytarabine
- B Transformation to diffuse large B-cell lymphoma with sudden clinical deterioration ✓
- C Transformation to Hodgkin lymphoma exclusively in del(13q) patients
- D Development of prolymphocytic leukaemia with CD5 loss
Correct answer: B. Transformation to diffuse large B-cell lymphoma with sudden clinical deterioration
Explanation
Richter's transformation (RT) is the transformation of CLL into an aggressive lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL, ~90% of cases), rarely Hodgkin lymphoma (~5%). It presents with rapid lymph node enlargement, fever, weight loss, elevated LDH, and worsening performance status. Median survival without treatment is 5–8 months. Clonally related RT (same clone as original CLL) has worse prognosis than unrelated RT. RT is associated with TP53 mutation and NOTCH1 mutation in CLL.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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