{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T11:47:01Z","timestamp":1790077621426,"version":"4.0.1"},"reference-count":32,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2016,1,5]],"date-time":"2016-01-05T00:00:00Z","timestamp":1451952000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2016,3]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Marginal zone lymphoma represents about 10% of all non\u2010Hodgkin lymphomas (<jats:styled-content style=\"fixed-case\">NHL<\/jats:styled-content>s). 33% of patients with acquired angioedema (<jats:styled-content style=\"fixed-case\">AAE<\/jats:styled-content>) due to acquired C1\u2010inhibitor (C1\u2010<jats:styled-content style=\"fixed-case\">INH<\/jats:styled-content>) deficiency (C1\u2010<jats:styled-content style=\"fixed-case\">INH<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">AAE<\/jats:styled-content>) have or will develop <jats:styled-content style=\"fixed-case\">NHL<\/jats:styled-content>s. C1\u2010<jats:styled-content style=\"fixed-case\">INH<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">AAE<\/jats:styled-content> is a rare condition. We report the follow\u2010up of 72 C1\u2010<jats:styled-content style=\"fixed-case\">INH<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">AAE<\/jats:styled-content> patients, followed for a median of 15\u00a0years (range 1\u201324). Median age was 71 (range 64\u201379) years; median age at onset of angioedema symptoms was 57\u00b75 (range 50\u201366) years and it was 63 [range 45\u201380) years at diagnosis]. Twenty patients were diagnosed with low\u2010grade non\u2010follicular B\u2010cell lymphomas (75% were splenic <jats:styled-content style=\"fixed-case\">MZL<\/jats:styled-content>), one with follicular and three with high\u2010grade lymphomas (two diffuse large B\u2010cell lymphomas and one mantle cell lymphoma). Fifteen <jats:styled-content style=\"fixed-case\">NHL<\/jats:styled-content>s were diagnosed at onset of <jats:styled-content style=\"fixed-case\">AAE<\/jats:styled-content> or thereafter (3\u00a0months to 7\u00a0years), eight had already been diagnosed at onset of angioedema. Two of 24 patients remain on watchful wait. Thirthen of 24 received chemotherapy, two received rituximab. Three underwent splenectomy. All 18 patients receiving therapy for <jats:styled-content style=\"fixed-case\">NHL<\/jats:styled-content> experienced post\u2010treatment reduction in <jats:styled-content style=\"fixed-case\">AAE<\/jats:styled-content> symptoms. Our study suggests that clonal B\u2010cell proliferation is the pathology underlying <jats:styled-content style=\"fixed-case\">AAE<\/jats:styled-content> leading to production of C1\u2010<jats:styled-content style=\"fixed-case\">INH<\/jats:styled-content>\u2010neutralizing autoantibodies and to <jats:styled-content style=\"fixed-case\">NHL<\/jats:styled-content>s. The post\u2010germinal centre origin of <jats:styled-content style=\"fixed-case\">NHL<\/jats:styled-content> suggests that immune stimulation may contribute to lymphomagenesis.<\/jats:p>","DOI":"10.1111\/bjh.13908","type":"journal-article","created":{"date-parts":[[2016,1,5]],"date-time":"2016-01-05T14:02:32Z","timestamp":1452002552000},"page":"902-908","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":51,"title":["High prevalence of splenic marginal zone lymphoma among patients with acquired C1 inhibitor deficiency"],"prefix":"10.1111","volume":"172","author":[{"given":"Roberto","family":"Castelli","sequence":"first","affiliation":[{"name":"Department of Biomedical and Clinical Sciences Luigi Sacco Luigi Sacco Hospital Milan University of Milan Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maddalena Alessandra","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Biomedical 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Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Davide","family":"Rossi","sequence":"additional","affiliation":[{"name":"Division of Haematology Department of Translational Medicine Amedeo Avogadro University of Eastern Piedmont Novara Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Cicardi","sequence":"additional","affiliation":[{"name":"Department of Biomedical and Clinical Sciences Luigi Sacco Luigi Sacco Hospital Milan University of Milan Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,1,5]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198705283162202"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1155\/2012\/638185"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.1998.16.8.2780"},{"key":"e_1_2_6_5_1","first-page":"349","article-title":"Acquired C1 esterase inhibitor 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