A 65-year-old man with CLL develops sudden worsening of lymphadenopathy, high fever, elevated LDH, and markedly elevated Ki-67 index on biopsy showing large transformed B cells. This transformation is called:

• A Blastic transformation
• B Reed-Sternberg conversion
• C Prolymphocytic transformation
• D Richter transformation ✓

Explanation

Richter transformation (Richter syndrome) refers to the transformation of CLL into an aggressive large B-cell lymphoma (usually diffuse large B-cell lymphoma) or, less commonly, Hodgkin lymphoma. It is associated with poor prognosis, systemic symptoms, markedly elevated LDH, and rapid lymph node enlargement. Blastic transformation occurs in CML to acute leukemia. Prolymphocytic transformation in CLL yields increased prolymphocytes but is a distinct entity from Richter syndrome.

Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.

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Written and medically reviewed by the StethoPrep medical team.