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Gastrointestinal Richter's syndrome.

N Brousse, P Solal-Celigny, A Herrera

    Human Pathology
    |August 1, 1985
    PubMed
    Summary

    A rare case of Richter's syndrome, a diffuse large cell lymphoma, developed in the stomach of a patient with chronic lymphocytic leukemia. This gut-localized presentation of Richter's syndrome has not been previously documented.

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    Area of Science:

    • Hematology
    • Oncology
    • Gastroenterology

    Background:

    • Chronic lymphocytic leukemia (CLL) is a lymphoid malignancy.
    • Richter's syndrome is the transformation of CLL into an aggressive lymphoma, typically diffuse large B-cell lymphoma (DLBCL).
    • Gastrointestinal involvement in Richter's syndrome is uncommon.

    Observation:

    • A patient with chronic lymphocytic leukemia developed diffuse large cell lymphoma in the stomach.
    • This represents a localized presentation of Richter's syndrome within the gastrointestinal tract.
    • Previous reports of Richter's syndrome have not described gut-specific localization.

    Findings:

    • Morphologic and immunologic studies indicated the diffuse large cell lymphoma originated from the same clonal proliferation (IgG lambda) as the CLL.

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  • The findings suggest a clonal evolution from CLL to DLBCL within the stomach.
  • This case expands the known clinical spectrum of Richter's syndrome.

    • Implications:

    • This case highlights the possibility of gastrointestinal manifestation of Richter's syndrome.
    • It underscores the importance of considering extranodal disease in CLL patients.
    • Further research may be needed to understand the mechanisms and frequency of gut-localized Richter's syndrome.