Academic Journal
Clinical characteristics of 512 eculizumab-naive paroxysmal nocturnal hemoglobinuria patients in China: a single-center observational study
| Title: | Clinical characteristics of 512 eculizumab-naive paroxysmal nocturnal hemoglobinuria patients in China: a single-center observational study |
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| Authors: | Yali Du, Yuan Yang, Chen Yang, Miao Chen, Bing Han |
| Source: | Hematology, Vol 27, Iss 1, Pp 113-121 (2022) |
| Publisher Information: | Taylor & Francis Group, 2022. |
| Publication Year: | 2022 |
| Collection: | LCC:Diseases of the blood and blood-forming organs |
| Subject Terms: | paroxysmal nocturnal hemoglobinuria, clinical manifestations, classic, bone marrow failure, complement inhibitors, Diseases of the blood and blood-forming organs, RC633-647.5 |
| More Details: | Objectives With large patient population and complement inhibitors naïve background, the characteristics patients with paroxysmal nocturnal hemoglobinuria (PNH) in China have not been well studied, especially for different subtypes. Methods We retrospectively reviewed patients with complete data who visited Peking Union Medical College Hospital (PUMCH) from 2009 to 2019 and had been followed up for more than 2 years. Results Five hundred and twelve patients were enrolled including 56.3% males and 43.7% females. The median age at disease onset was 33 (9∼80) years. Most were aged 21∼40 years (50.6%). 52.1%, 46.3% and 1.6% of the patients had classic PNH, bone marrow failure (BMF)/PNH and subclinical PNH, respectively. Symptoms of classic PNH were associated with hemolysis, whereas bleeding was more common in BMF/PNH patients. Classic PNH had higher survival rate, larger PNH clone size, higher lactate dehydrogenase (LDH) level and lower ferritin level than BMF/PNH. Although the rate of thrombosis was similar in the classic PNH and BMF/PNH (P = 0.66), those with BMF/PNH had higher chance of renal impairment (P |
| Document Type: | article |
| File Description: | electronic resource |
| Language: | English |
| ISSN: | 1607-8454 16078454 |
| Relation: | https://doaj.org/toc/1607-8454 |
| DOI: | 10.1080/16078454.2021.2022849 |
| Access URL: | https://doaj.org/article/9f89bf4a455c46d8b54c4bbd597ba4c5 |
| Accession Number: | edsdoj.9f89bf4a455c46d8b54c4bbd597ba4c5 |
| Database: | Directory of Open Access Journals |
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| ISSN: | 16078454 |
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| DOI: | 10.1080/16078454.2021.2022849 |
| Published in: | Hematology |
| Language: | English |