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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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Blood, 1 April 2003, Vol. 101, No. 7, pp. 2464-2472
REVIEW ARTICLE
Splenic marginal zone lymphoma
Vito Franco,
Ada Maria Florena, and
Emilio Iannitto
From the Istituto di Anatomia e Istologia Patologica
and Divisione di Ematologia con TMO, University of Palermo, Palermo,
Italy.
Splenic marginal zone lymphoma (SMZL) is a specific low-grade
small B-cell lymphoma that is incorporated in the World Health Organization 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 behavior are observed in a minority of patients. Molecular
and cytogenetic studies have shown heterogeneous results probably
because of the lack of standardized diagnostic criteria. To date, no
definitive therapy has been established. Therapeutic options include
treatment abstention, splenectomy, splenic irradiation, and chemotherapy.

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