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Primary splenic lymphoma: does it exist?

P G Gobbi1, G E Grignani, U Pozzetti

  • 1Dipartimento di Medicina Interna, Università degli Studi di Pavia, IRCCS Policlinico S. Matteo, Italy.

Haematologica
|May 1, 1994
PubMed
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Primary splenic lymphomas are increasingly reported. This study defines them restrictively, identifying two distinct types: splenic lymphoma with circulating villous lymphocytes and marginal-zone splenic lymphoma, with splenectomy as the primary treatment.

Area of Science:

  • Hematology
  • Oncology
  • Pathology

Background:

  • Primary splenic lymphomas are rare but increasingly reported.
  • Defining primary splenic lymphoma remains a subject of discussion.
  • A restrictive definition focusing on spleen and hilar lymph nodes is proposed to avoid overestimation.

Purpose of the Study:

  • To define primary splenic lymphoma restrictively.
  • To outline distinct clinicopathologic entities within primary splenic lymphoma.
  • To discuss therapeutic approaches for primary splenic lymphoma.

Main Methods:

  • Retrospective analysis of cases defined restrictively.
  • Histologic and immunologic characterization of lymphomas.
  • Clinical feature and prognostic factor assessment.

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Main Results:

  • Non-specific symptoms and low-to-intermediate grade histology are common.
  • Extranodal spread is the main unfavorable prognostic factor.
  • Two entities identified: splenic lymphoma with circulating villous lymphocytes and marginal-zone splenic lymphoma.

Conclusions:

  • Splenic lymphoma with circulating villous lymphocytes and marginal-zone splenic lymphoma are distinct entities.
  • Marginal-zone lymphoma cells share characteristics with MALT and B-monocytoid lymphomas.
  • Splenectomy remains the most effective therapy for primary splenic lymphomas.