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 Prepublished online as a Blood First Edition Paper on November 21, 2002; DOI 10.1182/blood-2002-07-2216.

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Submitted July 23, 2002
Accepted November 5, 2002

Splenic marginal zone lymphoma

Vito Franco*, Ada Maria Florena, and Emilio Iannitto

Istituto di Anatomia e Istologia Patologica, University of Palermo, Palermo, Italy
Divisione di Ematologia con TMO, University of Palermo, Palermo, Italy

* Corresponding author; email: vfranco{at}unipa.it.

Splenic marginal zone lymphoma (SMZL) is a specific low grade small B-cell lymphoma which is incorporated in the W.H.O. classification. Characteristic features are splenomegaly, moderate lymphocytosis with villous morphology, intrasinusoidal pattern of involvement of various organs, especially bone marrow, and relative indolent course. Tumor progression with increase of blastic forms and aggressive behaviour are observed in a minority of patients. Molecular and cytogenetic studies have shown heterogeneous results probably because of the lack of standardized diagnostic criteria. Yet there has been no definitive therapy established. Therapeutic options include treatment abstention, splenectomy, splenic irradiation and chemotherapy.


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