{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,23]],"date-time":"2025-09-23T12:45:00Z","timestamp":1758631500158},"reference-count":24,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2005,7,5]],"date-time":"2005-07-05T00:00:00Z","timestamp":1120521600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2005,8]]},"abstract":"<jats:p><jats:bold>Abstract:\u2002<\/jats:bold>  <jats:italic>Background<\/jats:italic>:\u2002Splenic marginal zone lymphoma (SMZL) is an infrequent B\u2010cell neoplasm that pursues an indolent course. Signs and symptoms, mostly related to hypersplenism, are successfully managed by splenectomy. However, the therapy of patients who are not fit for a surgical procedure or who relapse after splenectomy, is still an unsettled issue. <jats:italic>Patients and methods<\/jats:italic>:\u2002We report a phase\u2010II study on 16 patients with SMZL, three therapy na\u00efve and 13 pretreated, all showing systemic symptoms or progressive worsening of peripheral cytopenia, who were treated with pentostatin at a dose of 4\u2003mg\/m<jats:sup>2<\/jats:sup> every other week for 6\u201310\u2003wk. In relapsed patients, the median interval between diagnosis and treatment was 26\u2003month (range: 8\u201349). <jats:italic>Results<\/jats:italic>:\u2002Overall, 68% of the patients showed a clinical response. Two out three patients, who received pentostatin as first line therapy, attained a complete response (CR). One CR and seven minor or good haematological responses were recorded in relapsed patients. Treatment toxicity, mostly haematological, proved manageable. With a median follow\u2010up of 35\u2003month the median overall survival (OS) is 40\u2003month and the median progression free survival (PFS) is 18\u2003month. <jats:italic>Conclusion<\/jats:italic>:\u2002Our data show that pentostatin administered every other week has a good degree of activity in the treatment of SMZL and suggest that this schedule could be considered a possible therapeutic option for patients who are not fit for splenectomy or have relapsed.<\/jats:p>","DOI":"10.1111\/j.1600-0609.2005.00426.x","type":"journal-article","created":{"date-parts":[[2005,7,6]],"date-time":"2005-07-06T06:23:44Z","timestamp":1120631024000},"page":"130-135","source":"Crossref","is-referenced-by-count":24,"title":["Deoxycoformycin (pentostatin) in the treatment of splenic marginal zone lymphoma (SMZL) with or without villous 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