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Diffuse large B-cell lymphoma and its variants.

Mara Dominis1, Sonja Dzebro, Slavko Gasparov

  • 1Department of Pathology and Cytology, Merkur University Hospital, Zajceva 19, 10000 Zagreb, Croatia. mara.dominis@zg.tel.hr

Croatian Medical Journal
|October 29, 2002
PubMed
Summary

Diffuse large B-cell lymphoma (DLBCL) is a heterogeneous cancer. This study analyzed T-cell-rich and mediastinal variants, finding distinct survival outcomes and genetic profiles, highlighting the need for further stratification.

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Area of Science:

  • Hematopathology
  • Oncology
  • Molecular Biology

Background:

  • Diffuse large B-cell lymphoma (DLBCL) is a major subtype of non-Hodgkin lymphoma, comprising approximately 40% of adult cases.
  • DLBCL exhibits several morphological variants, including T-cell/histiocyte-rich and mediastinal (thymic) subtypes, each with unique characteristics.
  • Mediastinal DLBCL is an aggressive disease with a poor prognosis, potentially originating from thymic B cells.

Purpose of the Study:

  • To investigate the frequency and clinical behavior of T-cell/histiocyte-rich and mediastinal DLBCL variants.
  • To analyze survival outcomes and specific genetic features, such as bcl-2 gene rearrangement, in these DLBCL subtypes.
  • To emphasize the heterogeneity within DLBCL and the need for refined pathological and clinical classification.

Main Methods:

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  • Retrospective analysis of 101 patients diagnosed with DLBCL between 1997 and 2001.
  • Categorization of DLBCL cases into T-cell-rich B-cell lymphoma (17 patients) and mediastinal B-cell lymphoma (20 patients).
  • Evaluation of survival data and assessment for bcl-2 gene rearrangement in mediastinal DLBCL cases.

Main Results:

  • T-cell-rich B-cell lymphoma patients had a median survival of less than 20 months, unaffected by sex, bone marrow involvement, CD30 positivity, or histiocytic component.
  • Mediastinal B-cell lymphoma patients had a median survival of 21 months, with no significant impact from sex or lymph node necrosis.
  • No t(14;18) bcl-2 gene rearrangement was detected in the 20 mediastinal DLBCL cases studied.

Conclusions:

  • DLBCL remains a heterogeneous group of lymphoid neoplasms requiring further stratification.
  • T-cell-rich and mediastinal DLBCL variants present with distinct clinical behaviors and survival rates.
  • The absence of t(14;18) in mediastinal DLBCL suggests a different pathogenetic mechanism compared to some other DLBCL subtypes.