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Clinical implication of renal dysfunction during the clinical course in patients with paroxysmal nocturnal hemoglobinuria: a longitudinal analysis

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Abstract

Although renal dysfunction at the time of diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) is a risk factor for mortality, subsequent renal events can occur. The objective of this study was to identify clinical implication of renal dysfunction occurring during the disease course in PNH patients. One hundred one patients with a granulocyte clone size of > 10% were enrolled. Renal events were observed in 55 (54.5%) patients during a median follow-up of 94.2 months. Median time to first renal event from diagnosis of PNH was 79.3 months. Thromboembolism (TE) event and recurrent TE events were observed in 25 (24.8%) and 8 (7.9%) patients, respectively. The rate of recurrent TE was significantly higher in patients with renal events ≥ 2 compared with that in patients with renal event ≤ 1 (18.8% vs. 2.9%; P = 0.012). The rate of recurrent TE was significantly higher in patients with chronic kidney disease (CKD) + acute kidney disease (AKD) compared with the rest of the patients (27.3% vs. 5.6%; P = 0.040). CKD+AKD was the only independent risk factor for OS in multivariate analysis (hazard ratio 7.95, 95% CI 1.24–51.15, P = 0.029). Therefore, close monitoring of renal events in PNH patients during the entire clinical course is essential.

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Funding

This work was supported by the Korean Society of Hematology.

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Authors and Affiliations

  1. Division of Hematology, Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea

    Jin Seok Kim & June-Won Cheong

  2. Division of Hematology and Oncology, Department of Internal Medicine, Collage of Medicine, Ewha Womans University, Seoul, Republic of Korea

    Yeung-Chul Mun

  3. Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, School of Medicine, Sungkyunkwan University, Seoul, Republic of Korea

    Jun Ho Jang

  4. Department of Internal Medicine, School of Medicine, Chungnam National University Hospital, Chungnam National University, Daejeon, Republic of Korea

    Deog-Yeon Jo

  5. Division of Hematology, Department of Internal Medicine, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, 222 Banpo-daero, Seocho-gu, Seoul, 06591, Republic of Korea

    Jong Wook Lee

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  1. Jin Seok Kim
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on behalf of Aplastic Anemia Working Party of the Korean Society of Hematology

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Correspondence to Jin Seok Kim or Jong Wook Lee.

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All the authors stated that the study have been approved by the appropriate institutional review board and have been performed in accordance with the ethical standards as laid down in the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards. Since this is a retrospective study, formal informed consent is not required.

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Kim, J.S., Cheong, JW., Mun, YC. et al. Clinical implication of renal dysfunction during the clinical course in patients with paroxysmal nocturnal hemoglobinuria: a longitudinal analysis. Ann Hematol 98, 2273–2281 (2019). https://doi.org/10.1007/s00277-019-03735-6

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  • DOI: https://doi.org/10.1007/s00277-019-03735-6

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