Recent advances in the diagnosis and therapy of Richter's syndrome

Ronan Swords, John Bruzzi, Francis Giles*

*Corresponding author for this work

Research output: Contribution to journalReview article

20 Scopus citations

Abstract

Richter's syndrome (RS) denotes the development of aggressive lymphoma that arises in patients with chronic lymphocytic leukemia (CLL). Presenting features typically include a rapid clinical deterioration with fever in the absence of infection, progressive lymph node enlargement, and an elevation in serum LDH. Diagnostic biopsy of affected sites usually reveals large cell lymphomas; however, Hodgkin variant cases have been described. Richter's transformation occurs in approx 5% of CLL patients and may be associated with infection with Epstein-Barr virus (EBV). Chromosome 11 and 14 abnormalities have also been described as well as tumor suppressor gene defects involving p53, p21, and p27. Treatment options for these patients are limited and include combination chemotherapy with or without the addition of monoclonal antibodies and stem cell transplantation. Response to therapy is variable and generally short-lived. Median survival is usually in the order of 5-8 mo. More effective management for RS is needed as well as prognostic models that will identify CLL patients at risk of transformation. This review will address the current status of RS and deal with the pathophysiology, diagnostic approach, and treatment of this challenging disease.

Original languageEnglish (US)
Pages (from-to)17-32
Number of pages16
JournalMedical Oncology
Volume24
Issue number1
DOIs
StatePublished - Jun 1 2007

Keywords

  • Chronic lymphocytic leukemia
  • Epstein-Barr virus
  • Fluoro-18 deoxy-glucose
  • Gallium-67 citrate scintigraphy
  • Richter's syndrome
  • Transformation

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

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