A 70-year-old patient with CLL undergoes repeat biopsy after clinical deterioration. Biopsy now shows large B-cell lymphoma. This transformation carries a median survival of less than 12 months and is termed:
- A Richter transformation ✓
- B Beauchamp transformation
- C Sézary transformation
- D Doehle transformation
Correct answer: A. Richter transformation
Explanation
Richter transformation (or Richter syndrome) refers to the development of diffuse large B-cell lymphoma (DLBCL) in a patient with pre-existing CLL/SLL, occurring in approximately 2-5% of CLL patients. It is associated with rapid clinical deterioration, elevated LDH, and poor prognosis with a median survival under 12 months. The transformation typically involves NOTCH1 mutations and TP53 alterations in the CLL clone.
Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
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