{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T11:13:04Z","timestamp":1785928384280,"version":"3.56.0"},"reference-count":28,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2021,9,14]],"date-time":"2021-09-14T00:00:00Z","timestamp":1631577600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2022,1]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>We describe 36 patients with splenic marginal zone lymphoma (SMZL) with transformation (SMZL\u2010T), including 15 from a series of 84 patients with SMZL diagnosed at the Hospital Clinic of Barcelona (HCB) and 21 diagnosed with SMZL\u2010T in other centres. In the HCB cohort, the cumulative incidence of transformation at 5\u2009years was 15%. Predictors for transformation were cytopenias, hypoalbuminaemia, complex karyotype (CK) and both the Intergruppo Italiano Linfomi (ILL) and simplified Haemoglobin, Platelet count, lactate dehydrogenase (LDH) and extrahilar Lymphadenopathy (HPLL)\/ABC scores (<jats:italic>P<\/jats:italic>\u2009&lt;\u20090\u00b705). The only independent predictor for transformation in multivariate analysis was CK [hazard ratio (HR) 4\u00b7025, <jats:italic>P<\/jats:italic>\u2009=\u20090\u00b705]. Patients with SMZL\u2010T had a significantly higher risk of death than the remainder (HR 3\u00b789, <jats:italic>P<\/jats:italic>\u2009&lt;\u20090\u00b7001). Of the 36 patients with SMZL\u2010T, one developed Hodgkin lymphoma and 35 a diffuse large B\u2010cell lymphoma, 71% with a non\u2010germinal centre phenotype. The main features were B symptoms, lymphadenopathy, and high serum LDH. CK was observed in 12\/22 (55%) SMZL\u2010T and fluorescence <jats:italic>in\u00a0situ<\/jats:italic> hybridisation detected abnormalities of <jats:italic>MYC<\/jats:italic> proto\u2010oncogene, basic helix\u2010loop\u2010helix transcription factor (<jats:italic>MYC),<\/jats:italic> B\u2010cell leukaemia\/lymphoma 2 (<jats:italic>BCL2<\/jats:italic>) and\/or <jats:italic>BCL6<\/jats:italic> in six of 14 (43%). In all, 21 patients received immunochemotherapy, six chemotherapy, one radiotherapy and three splenectomy. The complete response (CR) rate was 61% and the median survival from transformation was 4\u00b792\u2009years. Predictors for a worse survival in multivariate analysis were high\u2010risk International Prognostic Index (HR 5\u00b7294, <jats:italic>P<\/jats:italic>\u2009=\u20090\u00b7016) and lack of CR (HR 2\u00b767, <jats:italic>P<\/jats:italic>\u2009&lt;\u20090\u00b7001).<\/jats:p>","DOI":"10.1111\/bjh.17815","type":"journal-article","created":{"date-parts":[[2021,9,14]],"date-time":"2021-09-14T07:27:57Z","timestamp":1631604477000},"page":"146-155","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["Clinico\u2010biological features and outcome of patients with splenic marginal zone lymphoma with histological transformation"],"prefix":"10.1111","volume":"196","author":[{"given":"Gabriela","family":"Bastidas\u2010Mora","sequence":"first","affiliation":[{"name":"Hematology Department and Hematopathology Section Pathology Department Hospital Cl\u00ednic of Barcelona Barcelona 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