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Related Experiment Videos

Primary digestive Richter's syndrome.

M Parrens1, B Sawan, P Dubus

  • 1Department of Pathology, Hôpital du Haut-Lévêque, Pessac, France. marie.parrens@chu-bordeaux.fr

Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc
|May 16, 2001
PubMed
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Digestive Richter's syndrome, a transformation of chronic lymphocytic leukemia (CLL) to diffuse large-cell lymphoma (DLCL) in the GI tract, shows a better prognosis than nodal cases. Combined treatment offers significant remission rates.

Area of Science:

  • Hematology
  • Oncology
  • Gastroenterology

Background:

  • Richter's syndrome is the transformation of chronic lymphocytic leukemia (CLL) into diffuse large-cell lymphoma (DLCL).
  • This transformation typically occurs in lymph nodes but can affect extranodal sites, including the gastrointestinal (GI) tract.
  • Digestive Richter's syndrome represents a distinct clinical entity with potentially different prognostic implications.

Purpose of the Study:

  • To investigate the clinical features, immunophenotype, and clonal origin of digestive Richter's syndrome.
  • To compare the prognosis of digestive Richter's syndrome with that of nodal Richter's syndrome.
  • To evaluate the efficacy of combined treatment modalities for digestive Richter's syndrome.

Main Methods:

  • Retrospective analysis of clinical data from six patients with digestive Richter's syndrome.

Related Experiment Videos

  • Immunohistological analysis of paired CLL and DLCL samples.
  • Polymerase chain reaction (PCR) for immunoglobulin heavy-chain gene rearrangement to assess clonality.

    • Main Results:

    • Histological examination confirmed GI tract involvement by DLCL of B-cell phenotype in all six patients.
    • PCR and sequencing demonstrated the same monoclonal immunoglobulin heavy-chain gene rearrangement between CLL and DLCL in two out of four patients, indicating transformation from the same clone.
    • Median survival was 22 months for five patients treated with chemotherapy.
    • Four patients achieved partial or complete remission with combined surgical resection and chemotherapy.

    Conclusions:

    • Digestive Richter's syndrome may have a more favorable prognosis compared to nodal Richter's syndrome.
    • Combined surgical resection and chemotherapy appear effective in achieving remission for digestive Richter's syndrome.
    • Further research is warranted to elucidate the specific mechanisms and optimize treatment strategies for this rare condition.