Cited 12 times in
Efficacy of eculizumab in paroxysmal nocturnal hemoglobinuria patients with or without aplastic anemia: prospective study of a Korean PNH cohort
DC Field | Value | Language |
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dc.contributor.author | 김진석 | - |
dc.date.accessioned | 2018-07-20T08:40:34Z | - |
dc.date.available | 2018-07-20T08:40:34Z | - |
dc.date.issued | 2017 | - |
dc.identifier.issn | 2287-979X | - |
dc.identifier.uri | https://ir.ymlib.yonsei.ac.kr/handle/22282913/161352 | - |
dc.description.abstract | BACKGROUND: Patients with paroxysmal nocturnal hemoglobinuria (PNH) often have concurrent aplastic anemia (AA). This study aimed to determine whether eculizumab-treated patients show clinical benefit regardless of concurrent AA. METHODS: We analyzed 46 PNH patients ≥18 years of age who were diagnosed by flow cytometry and treated with eculizumab for more than 6 months in the prospective Korean PNH registry. Patients were categorized into two groups: PNH patients with concurrent AA (PNH/AA, N=27) and without AA (classic PNH, N=19). Biochemical indicators of intravascular hemolysis, hematological laboratory values, transfusion requirement, and PNH-associated complications were assessed at baseline and every 6 months after initiation of eculizumab treatment. RESULTS: The median patient age was 46 years and median duration of eculizumab treatment was 34 months. Treatment with eculizumab induced rapid inhibition of hemolysis. At 6-month follow-up, LDH decreased to near normal levels in all patients; this effect was maintained until the 36-month follow-up regardless of concurrent AA. Transfusion independence was achieved by 53.3% of patients within the first 6 months of treatment and by 90.9% after 36 months of treatment. The mean number of RBC units transfused was significantly reduced, from 8.5 units during the 6 months prior to initiation of eculizumab to 1.6 units in the first 6 months of treatment, for the total study population; this effect was similar in both PNH/AA and classic PNH. CONCLUSION: This study demonstrated that eculizumab is beneficial in the management of patients with PNH/AA, similar to classic PNH. | - |
dc.description.statementOfResponsibility | open | - |
dc.language | English | - |
dc.publisher | Korea Society of Hematology | - |
dc.relation.isPartOf | BLOOD RESEARCH | - |
dc.rights | CC BY-NC-ND 2.0 KR | - |
dc.rights | https://creativecommons.org/licenses/by-nc-nd/2.0/kr/ | - |
dc.title | Efficacy of eculizumab in paroxysmal nocturnal hemoglobinuria patients with or without aplastic anemia: prospective study of a Korean PNH cohort | - |
dc.type | Article | - |
dc.contributor.college | College of Medicine | - |
dc.contributor.department | Dept. of Internal Medicine | - |
dc.contributor.googleauthor | Chul Won Choi | - |
dc.contributor.googleauthor | Jun Ho Jang | - |
dc.contributor.googleauthor | Jin Seok Kim | - |
dc.contributor.googleauthor | Deog-Yeon Jo | - |
dc.contributor.googleauthor | Je-Hwan Lee | - |
dc.contributor.googleauthor | Sung-Hyun Kim | - |
dc.contributor.googleauthor | Yeo-Kyeoung Kim | - |
dc.contributor.googleauthor | Jong-Ho Won | - |
dc.contributor.googleauthor | Joo Seop Chung | - |
dc.contributor.googleauthor | Hawk Kim | - |
dc.contributor.googleauthor | Jae Hoon Lee | - |
dc.contributor.googleauthor | Min Kyoung Kim | - |
dc.contributor.googleauthor | Hyeon-Seok Eom | - |
dc.contributor.googleauthor | Shin Young Hyun | - |
dc.contributor.googleauthor | Jeong-A Kim | - |
dc.contributor.googleauthor | Jong Wook Lee | - |
dc.identifier.doi | 10.5045/br.2017.52.3.207 | - |
dc.contributor.localId | A01017 | - |
dc.relation.journalcode | J00346 | - |
dc.identifier.eissn | 2288-0011 | - |
dc.identifier.pmid | 29043236 | - |
dc.subject.keyword | Aplastic anemia | - |
dc.subject.keyword | Complement C5 inhibitor | - |
dc.subject.keyword | Eculizumab | - |
dc.subject.keyword | Paroxysmal nocturnal hemoglobinuria | - |
dc.contributor.alternativeName | Kim, Jin Seok | - |
dc.contributor.affiliatedAuthor | Kim, Jin Seok | - |
dc.citation.volume | 52 | - |
dc.citation.number | 3 | - |
dc.citation.startPage | 207 | - |
dc.citation.endPage | 211 | - |
dc.identifier.bibliographicCitation | BLOOD RESEARCH, Vol.52(3) : 207-211, 2017 | - |
dc.identifier.rimsid | 61271 | - |
dc.type.rims | ART | - |
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