dc.contributor.author | Berentsen, Sigbjørn | |
dc.contributor.author | Barcellini, Wilma | |
dc.contributor.author | D'Sa, Shirley | |
dc.contributor.author | Randen, Ulla | |
dc.contributor.author | Tvedt, Tor Henrik Anderson | |
dc.contributor.author | Fattizzo, Bruno | |
dc.contributor.author | Haukås, Einar | |
dc.contributor.author | Kell, Megan | |
dc.contributor.author | Brudevold, Robert | |
dc.contributor.author | Dahm, Anders Erik Astrup | |
dc.contributor.author | Dalgaard, Jakob | |
dc.contributor.author | Frøen, Hege | |
dc.contributor.author | Hallstensen, Randi | |
dc.contributor.author | Jæger, Pernille Horgmo | |
dc.contributor.author | Hjorth-Hansen, Henrik | |
dc.contributor.author | Malecka, Agnieszka Maria | |
dc.contributor.author | Oksman, Markku | |
dc.contributor.author | Rolke, Jürgen | |
dc.contributor.author | Sekhar, Mallika | |
dc.contributor.author | Sørbø, Jon Hjalmar | |
dc.contributor.author | Tjønnfjord, Eirik | |
dc.contributor.author | Tsykunova, Galina | |
dc.contributor.author | Tjønnfjord, Geir Erland | |
dc.date.accessioned | 2021-04-22T08:03:33Z | |
dc.date.available | 2021-04-22T08:03:33Z | |
dc.date.created | 2021-01-06T12:55:34Z | |
dc.date.issued | 2020 | |
dc.identifier.citation | Blood. 2020, 136 (4), 480-488. | en_US |
dc.identifier.issn | 0006-4971 | |
dc.identifier.uri | https://hdl.handle.net/11250/2739004 | |
dc.description.abstract | We retrospectively studied 232 patients with cold agglutinin disease (CAD) at 24 centers in 5 countries. In Norway and a northern region of Italy, the study was close to being population-based. For the first time, we demonstrate fourfold differences between cold and warmer climates regarding prevalence (20 vs 5 cases/million) and incidence (1.9 vs 0.48 cases/million per year). Mean baseline hemoglobin level was 9.3 g/dL, but 27% had hemoglobin <8 g/dL. Identification of typical features of CAD-associated lymphoproliferative disorder in the bone marrow was greatly increased by centralized biopsy assessment. CAD seems to be associated with a slightly increased risk of venous thrombosis. This work includes a follow-up study of therapies, focusing on the long-term outcomes of the rituximab plus bendamustine and rituximab plus fludarabine regimens. Rituximab plus bendamustine therapy resulted in responses in 35 (78%) of 45 patients; 24 (53%) achieved complete response. Interestingly, these rates were still higher than observed in the original (2017) prospective trial, and we also found a shift toward deeper responses with time. This is explained by the prolonged time to response seen in many patients, probably related to long-lived plasma cells. In patients responding to rituximab-bendamustine, median response duration was not reached after 88 months, and estimated 5-year sustained remission was 77%. The regimen appeared safe regarding late-occurring malignancies. Rituximab plus fludarabine therapy seems to carry a higher risk of long-term adverse effects. | en_US |
dc.language.iso | eng | en_US |
dc.publisher | American Society of Hematology | en_US |
dc.title | Cold agglutinin disease revisited: a multinational, observational study of 232 patients | en_US |
dc.type | Peer reviewed | en_US |
dc.type | Journal article | en_US |
dc.description.version | publishedVersion | en_US |
dc.source.pagenumber | 480-488 | en_US |
dc.source.volume | 136 | en_US |
dc.source.journal | Blood | en_US |
dc.source.issue | 4 | en_US |
dc.identifier.doi | 10.1182/blood.2020005674 | |
dc.identifier.cristin | 1866298 | |
dc.description.localcode | This article will not be available due to copyright restrictions © 2020 by American Society of Hematology. | en_US |
cristin.ispublished | true | |
cristin.fulltext | original | |
cristin.qualitycode | 2 | |