{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,11]],"date-time":"2025-09-11T19:33:41Z","timestamp":1757619221862,"version":"3.44.0"},"reference-count":32,"publisher":"Springer Science and Business Media LLC","issue":"9","license":[{"start":{"date-parts":[[2025,7,21]],"date-time":"2025-07-21T00:00:00Z","timestamp":1753056000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/deed.de"},{"start":{"date-parts":[[2025,7,21]],"date-time":"2025-07-21T00:00:00Z","timestamp":1753056000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/deed.de"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Innere Medizin"],"published-print":{"date-parts":[[2025,9]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title>\n          <jats:sec>\n            <jats:title>Hintergrund<\/jats:title>\n            <jats:p>Die K\u00e4lteagglutininerkrankung (\u201ecold agglutinin disease\u201c [CAD]) ist eine seltene, aber klinisch eindrucksvolle Erkrankung mit hohem Leidensdruck und einem Risiko f\u00fcr schwerwiegende thromboembolische Komplikationen.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Ziel<\/jats:title>\n            <jats:p>Der vorliegende \u00dcbersichtsbeitrag soll eine pr\u00e4gnante, klinisch orientierte Zusammenfassung des aktuellen Kenntnisstands zur Erkrankung und zu den Therapieoptionen bieten.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Ergebnisse<\/jats:title>\n            <jats:p>Die Diagnose erfordert den Nachweis einer chronischen H\u00e4molyse, die Detektion von C3d im monospezifischen direkten Coombs- bzw. Antiglobulintest (DAT), den Nachweis von K\u00e4lteagglutininen mit einem Titer \u2265\u202f1:64 bei 4\u202f\u00b0C und den Ausschluss einer malignen Erkrankung oder relevanten Infektion. Therapeutische Optionen waren bislang die Vermeidung von K\u00e4lte, eine ad\u00e4quate Hydratation bei h\u00e4molytischen Krisen, eine Thromboseprophylaxe sowie eine immunsuppressive Therapie mit Rituximab und\/oder Zytostatika. Die einzige zugelassene Therapie ist eine Komplementinhibition mit Sutimlimab. Die H\u00e4molyse spricht auf eine Inhibition des klassischen Komplementwegs durch den Anti-C1s-Antik\u00f6rper Sutimlimab innerhalb von wenigen Tagen mit einem Abfall der H\u00e4molyseparameter und einer Verbesserung der Fatigue an. Die Therapie erfordert einen vollumf\u00e4nglichen Impfschutz gegen bekapselte Bakterien und gegebenenfalls eine \u00fcberbr\u00fcckende antibiotische Prophylaxe, bis dieser erreicht ist. Eine supportive Therapie, beispielsweise die Gabe von Fols\u00e4ure, Vitamin\u00a0B12 und Eisen bei Mangel, oder auch eine Kombination mit weiteren Therapiestrategien sollte gegebenenfalls erwogen werden. Daten zum Einsatz von Sutimlimab beim sekund\u00e4ren K\u00e4lteagglutininsyndrom (CAS) liegen bislang nicht vor.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Schlussfolgerung<\/jats:title>\n            <jats:p>Die Kenntnis der spezifischen Klinik und der Laborver\u00e4nderungen bei einer K\u00e4lteagglutininerkrankung mit der fr\u00fchzeitigen Einleitung einer spezifischen und zielgerichteten Therapie oder aber auch mit der \u00dcberweisung an ein spezialisiertes Zentrum hat die Prognose der Erkrankung in den letzten Jahren deutlich verbessert und den Leidensdruck der Patienten reduziert.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1007\/s00108-025-01926-0","type":"journal-article","created":{"date-parts":[[2025,7,21]],"date-time":"2025-07-21T18:03:24Z","timestamp":1753121004000},"page":"986-996","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["K\u00e4lteagglutininerkrankung","Cold agglutinin disease (CAD)"],"prefix":"10.1007","volume":"66","author":[{"given":"Alexander","family":"R\u00f6th","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kersten","family":"Borchert","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carla","family":"Dorn","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Moritz","family":"Kleemi\u00df","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sixten","family":"K\u00f6rper","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephanie","family":"Mayer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Philippe","family":"Schafhausen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karin G.","family":"Schrenk","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter","family":"Bramlage","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Frauke","family":"Theis","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2025,7,21]]},"reference":[{"issue":"5","key":"1926_CR1","doi-asserted-by":"publisher","first-page":"361","DOI":"10.1080\/10245330701445392","volume":"12","author":"S Berentsen","year":"2007","unstructured":"Berentsen\u00a0S, Beiske\u00a0K, Tjonnfjord\u00a0GE (2007) Primary chronic cold agglutinin disease: an update on pathogenesis, clinical features and therapy. 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