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

Ronan T Swords, John Bruzzi, Francis Giles

Research output: Contribution to journalArticle

19 Citations (Scopus)

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

Fingerprint

B-Cell Chronic Lymphocytic Leukemia
Lymphoma
Chromosomes, Human, Pair 14
Chromosomes, Human, Pair 11
Epstein-Barr Virus Infections
Stem Cell Transplantation
Therapeutics
Combination Drug Therapy
Tumor Suppressor Genes
Hodgkin Disease
Fever
Lymph Nodes
Monoclonal Antibodies
Biopsy
Survival
Infection
Serum

Keywords

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

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

Cite this

Recent advances in the diagnosis and therapy of Richter's syndrome. / Swords, Ronan T; Bruzzi, John; Giles, Francis.

In: Medical Oncology, Vol. 24, No. 1, 01.06.2007, p. 17-32.

Research output: Contribution to journalArticle

Swords, Ronan T ; Bruzzi, John ; Giles, Francis. / Recent advances in the diagnosis and therapy of Richter's syndrome. In: Medical Oncology. 2007 ; Vol. 24, No. 1. pp. 17-32.
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