{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T01:38:20Z","timestamp":1789609100955,"version":"build-2803163510"},"reference-count":53,"publisher":"Oxford University Press (OUP)","issue":"12","license":[{"start":{"date-parts":[[2024,4,24]],"date-time":"2024-04-24T00:00:00Z","timestamp":1713916800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"National Plan for Rare Diseases of the French Ministry of Health"},{"DOI":"10.13039\/501100009243","name":"Direction G\u00e9n\u00e9rale de l\u2019offre de Soins","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100009243","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,11,27]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Carfilzomib, a new proteasome inhibitor indicated for patients with relapsed\/refractory myeloma, has been associated with cases of thrombotic microangiopathy (CFZ-TMA). The role of variants in the complement alternative pathway and therapeutic potential of complement blockade with eculizumab remain to be determined.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>We report 37 cases of CFZ-TMA recorded in the French reference center for TMA with their clinical characteristics, genetic analysis and outcome according to treatments.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>A trigger was identified in more than half of cases, including eight influenza and five severe acute respiratory syndrome coronavirus-2 cases. All patients presented with acute kidney injury (AKI) [KDIGO stage 3 in 31 (84%) patients] while neurological (n\u00a0=\u00a013, 36%) and cardiac (n\u00a0=\u00a07, 19%) damage were less frequent. ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type I repeats-13) and complement activity were normal (n\u00a0=\u00a028 and 18 patients tested) and no pathogenic variant in the alternative complement pathway was found in 7 patients tested. TMA resolved in most (n\u00a0=\u00a034, 94%) patients but 12 (44%) still displayed stage 3 AKI at discharge. Nineteen (51%) patients were treated with therapeutic plasma exchange, 14 (38%) patients received corticosteroids and 18 (50%) were treated with eculizumab. However, none of these treatments demonstrated a significant impact on outcomes.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>This study is the largest case series of CFZ-TMA since its approval in 2012. Patients present with severe AKI and experience frequent sequelae. Complement variants and blockade therapy do not seem to play a role in the pathophysiology and prognosis of the disease.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ndt\/gfae096","type":"journal-article","created":{"date-parts":[[2024,4,23]],"date-time":"2024-04-23T21:02:58Z","timestamp":1713906178000},"page":"2067-2078","source":"Crossref","is-referenced-by-count":8,"title":["Carfilzomib-associated thrombotic microangiopathy: clinical features and outcomes"],"prefix":"10.1093","volume":"39","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5278-8966","authenticated-orcid":false,"given":"Adrien","family":"Joseph","sequence":"first","affiliation":[{"name":"Service de M\u00e9decine intensive r\u00e9animation, H\u00f4pital Saint Louis, Assistance Publique des H\u00f4pitaux de Paris , Paris ,","place":["France"]},{"name":"Centre de R\u00e9f\u00e9rence des Microangiopathies Thrombotiques (CNR-MAT), Assistance Publique des H\u00f4pitaux de Paris , Paris 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