Mantle cell lymphoma: a lymphoproliferative disorder associated with aberrant function of the cell cycle

J Garcia-Conde1, F Cabanillas

  • 1Lymphoma Section, MD Anderson Cancer Center, Houston, TX 77030, USA.

Leukemia
|June 1, 1996
PubMed

Insights

Mantle cell lymphoma, a B cell disorder, is defined by t(11;14) translocation and CD5+/CD20+ immunophenotype. The diffuse subtype indicates a poor prognosis, with limited treatment efficacy.

Area of Science:

  • Hematology
  • Oncology
  • Molecular Biology

Background:

  • Mantle cell lymphoma (MCL) is a B cell lymphoproliferative neoplasm.
  • It is characterized by the t(11;14) chromosomal translocation involving the cyclin D1 gene (CCND1).
  • Immunophenotypically, MCL typically expresses CD5 and CD20, while being negative for CD23.

Purpose of the Study:

  • To elucidate the cytogenetic, molecular, and immunophenotypic characteristics of Mantle Cell Lymphoma.
  • To describe the different histological patterns and their prognostic implications.
  • To review the impact of current therapeutic regimens and explore experimental treatments.

Main Methods:

  • Cytogenetic analysis to identify the t(11;14) translocation.
  • Molecular studies to confirm Bcl-1/PRAD-1 gene involvement and cyclin D1 overexpression.
  • Immunohistochemistry to determine antigen expression (CD5, CD20, CD23).
  • Histopathological examination of tissue samples to classify patterns (diffuse, mantle zone, nodular).

Main Results:

  • The t(11;14) translocation leading to cyclin D1 overexpression drives cell proliferation by affecting the Rb protein pathway.
  • The diffuse histological pattern is the most common and associated with a poor prognosis.
  • Standard anthracycline-based chemotherapy regimens show limited impact on survival for nodular or diffuse MCL.
  • Five-year survival rates are below 30% in some series.

Conclusions:

  • Mantle cell lymphoma is a distinct entity with specific molecular drivers of proliferation.
  • Histological patterns significantly influence patient outcomes.
  • Current treatment strategies have limitations, necessitating further research into intensive therapies and transplantation.

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