Matches in SemOpenAlex for { <https://semopenalex.org/work/W2776262336> ?p ?o ?g. }
Showing items 1 to 99 of
99
with 100 items per page.
- W2776262336 endingPage "73" @default.
- W2776262336 startingPage "69" @default.
- W2776262336 abstract "ObjectiveTo explore the benefits of adding eculizumab for the treatment of refractory autoimmune thrombotic thrombocytopenic purpura (iTTP) with complement dysregulation.Patients and MethodsFrom January 1, 2014, through July 1, 2017, we identified patients with iTTP defined by ADAMTS13 (disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) levels less than 5% and the presence of ADAMTS13 inhibitor. Patients who progressed after receiving standard of care management for iTTP were subjected to a comprehensive evaluation to look for evidence of complement activation. Herein, we share our single-institute experience regarding the clinical course and treatment algorithm for 3 patients with refractory iTTP.ResultsAll the patients had clinical deterioration despite treatment with plasma exchange, corticosteroids, rituximab, and vincristine, which prompted us to look for evidence of complement activation and associated genetic mutations. Complement-related genetic aberrations were present in all 3 patients, who had had different degrees of complement activation. The first 2 patients did not benefit from eculizumab when treatment was started before complete clearance of inhibitors to ADAMTS13. However, they had durable remissions when eculizumab was introduced after clearance of ADAMTS13 inhibitors. The third patient started eculizumab therapy after inhibitor levels were undetectable.ConclusionWe found eculizumab therapy to be effective in all 3 patients. However, its efficacy was prominent only after clearance of antibodies against ADAMTS13 via therapeutic plasma exchange." @default.
- W2776262336 created "2018-01-05" @default.
- W2776262336 creator A5042487436 @default.
- W2776262336 creator A5048028937 @default.
- W2776262336 creator A5076152697 @default.
- W2776262336 creator A5080169097 @default.
- W2776262336 creator A5081293249 @default.
- W2776262336 date "2018-03-01" @default.
- W2776262336 modified "2023-10-16" @default.
- W2776262336 title "Complement Regulatory Genetic Mutations in the Setting of Autoimmune Thrombotic Thrombocytopenic Purpura: A Case Series" @default.
- W2776262336 cites W1963924977 @default.
- W2776262336 cites W1977035416 @default.
- W2776262336 cites W1989118126 @default.
- W2776262336 cites W2029886021 @default.
- W2776262336 cites W2038181025 @default.
- W2776262336 cites W2039724947 @default.
- W2776262336 cites W2054101977 @default.
- W2776262336 cites W2067053006 @default.
- W2776262336 cites W2078450039 @default.
- W2776262336 cites W2098315002 @default.
- W2776262336 cites W214371270 @default.
- W2776262336 cites W2313540521 @default.
- W2776262336 cites W2340760838 @default.
- W2776262336 cites W2772085158 @default.
- W2776262336 doi "https://doi.org/10.1016/j.mayocpiqo.2017.11.004" @default.
- W2776262336 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/6124327" @default.
- W2776262336 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/30225434" @default.
- W2776262336 hasPublicationYear "2018" @default.
- W2776262336 type Work @default.
- W2776262336 sameAs 2776262336 @default.
- W2776262336 citedByCount "6" @default.
- W2776262336 countsByYear W27762623362019 @default.
- W2776262336 countsByYear W27762623362020 @default.
- W2776262336 countsByYear W27762623362022 @default.
- W2776262336 crossrefType "journal-article" @default.
- W2776262336 hasAuthorship W2776262336A5042487436 @default.
- W2776262336 hasAuthorship W2776262336A5048028937 @default.
- W2776262336 hasAuthorship W2776262336A5076152697 @default.
- W2776262336 hasAuthorship W2776262336A5080169097 @default.
- W2776262336 hasAuthorship W2776262336A5081293249 @default.
- W2776262336 hasBestOaLocation W27762623361 @default.
- W2776262336 hasConcept C111684460 @default.
- W2776262336 hasConcept C121332964 @default.
- W2776262336 hasConcept C126322002 @default.
- W2776262336 hasConcept C142424586 @default.
- W2776262336 hasConcept C159654299 @default.
- W2776262336 hasConcept C203014093 @default.
- W2776262336 hasConcept C2777637130 @default.
- W2776262336 hasConcept C2777878052 @default.
- W2776262336 hasConcept C2777991916 @default.
- W2776262336 hasConcept C2778585876 @default.
- W2776262336 hasConcept C2779134260 @default.
- W2776262336 hasConcept C2780653079 @default.
- W2776262336 hasConcept C2780921031 @default.
- W2776262336 hasConcept C71924100 @default.
- W2776262336 hasConcept C87355193 @default.
- W2776262336 hasConcept C89560881 @default.
- W2776262336 hasConcept C90924648 @default.
- W2776262336 hasConceptScore W2776262336C111684460 @default.
- W2776262336 hasConceptScore W2776262336C121332964 @default.
- W2776262336 hasConceptScore W2776262336C126322002 @default.
- W2776262336 hasConceptScore W2776262336C142424586 @default.
- W2776262336 hasConceptScore W2776262336C159654299 @default.
- W2776262336 hasConceptScore W2776262336C203014093 @default.
- W2776262336 hasConceptScore W2776262336C2777637130 @default.
- W2776262336 hasConceptScore W2776262336C2777878052 @default.
- W2776262336 hasConceptScore W2776262336C2777991916 @default.
- W2776262336 hasConceptScore W2776262336C2778585876 @default.
- W2776262336 hasConceptScore W2776262336C2779134260 @default.
- W2776262336 hasConceptScore W2776262336C2780653079 @default.
- W2776262336 hasConceptScore W2776262336C2780921031 @default.
- W2776262336 hasConceptScore W2776262336C71924100 @default.
- W2776262336 hasConceptScore W2776262336C87355193 @default.
- W2776262336 hasConceptScore W2776262336C89560881 @default.
- W2776262336 hasConceptScore W2776262336C90924648 @default.
- W2776262336 hasIssue "1" @default.
- W2776262336 hasLocation W27762623361 @default.
- W2776262336 hasLocation W27762623362 @default.
- W2776262336 hasLocation W27762623363 @default.
- W2776262336 hasLocation W27762623364 @default.
- W2776262336 hasOpenAccess W2776262336 @default.
- W2776262336 hasPrimaryLocation W27762623361 @default.
- W2776262336 hasRelatedWork W1808843406 @default.
- W2776262336 hasRelatedWork W1980805976 @default.
- W2776262336 hasRelatedWork W2275639500 @default.
- W2776262336 hasRelatedWork W2573372604 @default.
- W2776262336 hasRelatedWork W2776262336 @default.
- W2776262336 hasRelatedWork W2885610407 @default.
- W2776262336 hasRelatedWork W2978019642 @default.
- W2776262336 hasRelatedWork W4286373062 @default.
- W2776262336 hasRelatedWork W4286839279 @default.
- W2776262336 hasRelatedWork W4294096092 @default.
- W2776262336 hasVolume "2" @default.
- W2776262336 isParatext "false" @default.
- W2776262336 isRetracted "false" @default.
- W2776262336 magId "2776262336" @default.
- W2776262336 workType "article" @default.